Anthony Gordon Butler, MBA., ABEng., AGIS., AMIPD. A thesis submitted to the University of ...

A comprehensive and lOngitudinaLstuqy d logical, the social, economic epi emio of implications of and psychological dystonia within the popula ti .on of the North. East. of England Anthony Gordon Butler, MBA., ABEng., AGIS., AMIPD.

A thesis submitted to the University of Newcastle-upon-Tyne in support of an application for the Degree of Doctor of Philosophy to the Faculty of Medicine NEWCASTLE UNIVERSITY ----------------------------099 21857 4 ----------------------------

LIBRARY

Dystonia -A comprehensiveand longitudinal study in the North East of England

CONTENTS PageNo. i-vi

Index of pages Publications Tables and Charts Abstract

PART ONE Chapter I

vii ix Xiv

: INTRODUCTION Introduction

and Aim of the Research

Whatis Dystonia? Whatis the aimof this research? Why wasit necessary ? Chapter 2

Background How did this researchcomeabout? Why wasit donein the wayit was? Whendid it start?

PART TWO Chapter 3

DYSTONIA : Literature

Review

5

GeneralReview InternationalClassification of Diseases TheReadCodes EpidemiologicalSurveysof Dystonia Disabilityin General The SocialModel of Disability Chapter 4:

Classification

9

Aetiology Distribution Chapter 5:

Previous Epidemiologies GeneralReview Resultsof previousepiderniologies TheNorth Eastof England(includingCumbria)

i

14

Dystonia -A comprehensiveand longitudinal study in the North East of England

Chapter 6:

Treatments

19

Conditionswith a SpecificRational Therapy Surgery and OrthopaedicProcedures Drug Treatments The Introduction of Botulinum Toxin Therapy

PART THREE: Chapter 7:

METHODOLOGY

Awareness Raising

27

Twelve AwarenessRaisingEvents in the UK Four AwarenessRaisingEvents abroad

Chapter 8:

Data Collection and Instruments

29

Data Collection Instruments Quality of Life and Health State Questionnaire Demographicand Dystonic Profile Questionnaire TDS Questionnaire Demographic ChangesQuestionnaire EnvironmentalQuestionnaire Diagnostic Questionnaire Dystonia Nurse Practitioner Project Clinical Information Form Validation

Chapter 9:

The Cost-Utility Analysis

37

TheEuroQoIQuestionnaire The SF36HealthStatusQuestionnaire TheDystoniaSelf-Perception Questionnaire Chapter 10 : The Epidemiology of Primary Dystonia

40

Chapter 11 : Psycho-Social Research

42

Clinical Profile of Dystonia Questionnaire Torticollis Questionnaire Living with Dystonia Questionnaire Impact of Dystonia Questionnaire Primary Carer's Questionnaire

Chapter 12 : Socio-Economic Research

ii

45

Dystonia -A comprehensiveand longitudinal study in the North East of England

Chapter 13 : The Dystonia Nurse Practitioner

Project

48

The Inclusion Criteria The Exclusion Criteria

Chapter 14 : Data Input and Coding Frame

48

ESD EpidemiologicalSurvey of Dystonia CUA Cost Utility Analysis EPD Epidemiology of Primary Dystonia DNP Dystonia Nurse Practitioner PSR Psycho-SocialResearch 1171) Impact of Focal Dystonia on Musicians SER Socio-EconomicResearch PatientNumbering System SyntaxFiles SPSSCoding Frame

PART FOUR

ANALYSIS OF FINDINGS :

Chapter 15 : Epidemiology, Prevalence and Incidence

50

Chapter 16 : Diagnoses, previous and current

52

Diagnosis Cý

Chapter 17 : Treatments, previous and current

56

Botulinum Toxin Therapyand Side Effects Previous Treatments Medications Prescriptions

Chapter 18 : Comorbidity

and The Quality of Life

64

Comorbidity Major Illnesses Movements Pain Levels SpontaneousImprovement Quality of Life Results

Chapter 19 : Genetics

71

iii

Dystonia -A comprehensiveand longitudinal study in the North East of England

Chapter 20 : Social Implications

73

Social and Economic Groupings Depression,Anxiety and Pain Social Isolation Marital Status,Children and Hereditary Time off work Travel to and from hospital Major events Living Standards

Chapter 21

Economic Implications :

83

Employment Status Financial Income Economic Benefits

Chapter 22

: Psychological Profiles

88

PsychiatricProblems Living with Dystonia Questionnaire Disabling, Uncomfortableand Disfigurement Body Concept Scales The Beck DepressionInventory Self EsteemScores The Impact of Dystonia The AcceptanceStages The Primary Carer's Questionnaire Carer's PersonalStatements

Chapter 23

: Environmental

Factors

Extremities The Thyroid Gland Life Patterns Smoking Gas Appliances External Influences: Sound,Light and Drinking Water SpasmTests Exposure to Chemicals Allergy Problems Food and Drink EnvironmentalFactors The Analysis of Conduct of Research

iv

99

Dystonia -A comprehensiveand longitudinal study in the North East of England

Chapter 24

: The Dystonia Society

III

Contact Counselling TDS Membership The Welfare Benefits Service

Chapter 25

: The Spasmodic Torticollis Questionnaire

116

Position and Direction of the Head GesteAntagoniste Which handdo you use ? Where and how do you placeyour hand ?

Chapter 26

: The Outreach Nurse Practitioner Project

118

The Pilot Scheme The Interviews Application of the Questionnaires Comparisonbetweenthe two groups Input and analysisof data The results

Chapter 27

: The Impact of Focal Dystonia on the Working Life of Musicians

140

Case History No I Case History No 2 Case History No 3

PART FIVE Chapter 28

: DISCUSSION

: Epidemiology

143

Distribution SpasmodicDysphonia

Chapter 29

: Diagnosis

150

Chapter 30

: Treatment

152

Chapter 31

: Genetics

154

V

Dystonia -A comprehensiveand longitudinal study in the North East of England

Chapter 32 : Quality of Life

155-

Chapter 33

157

Social : and Economic Implications Economic Consequences Social Consequences

Chapter 34

: Psychological Profiles

162

Chapter 35

: Environmental

164

Chapter 36

Conclusion :

Factors

166

PART SIX: APPENDICES (separately bound) Appendix A: Acknowledgments The researchteamson the various projects Acknowledgementof the individual contributions Acknowledgementof the financial contributions

Appendix B : Instruments A completeset of questionnaires

Appendix C:

SPSS Coding File The Locoscript version of the Coding Frame

Appendix D : Syntax Files 6 SPSSSyntaxFiles

Appendix E : Other documentation Copy of the initial letter from the Doctor in the CUA Copy of the ConsentForm Copy of the Clinical Information Form Copy of the Clinical AnalysisForm

Appendix F : Verbatim Answers Appendix G: References

%i

Dystonia -A comprehensiveand longitudinal study in the North East of England

PUBLICATIONS I The following abstracts and publications have been published since the start of the form and part of the ongoing relationship with other contributors to the research None in the projects. research of specific material used previous publications is overall in this research, except where noted. quoted

Butler A. G. (1995) The socio-economic implications of dystonia. The Dystonia SocietyNewsletter. ; 19 ; 4-5 and 20 ; 4-5 and 21 ; 5-6. London.,TDS. Butler A. G. (1996) The social and economic implications of dystonia. Ellropeall Journal ofNeurology ;3: 79. Butler A. G. (1997) The epidemiology of Spasmodic Torticollis in North East England. In: 7he National Spasmodic Torticollis Association Annual Symposiumin Nashville, Tennesseeon 9th November 1997. Butler A. G, Duffey P. O.F. (1996a) The epidemiological survey of dystonia in the North East of England. Europewt Jounial ofNeurology: 3: 28

Butler A. G, Duffey P.O.F. (1996b) An epidemiological survey of dystonia at Darlington Memorial Hospital. EuropeanJournal ofNeurology : 3: 79 Butler A. G, Duffey P.O.F. (1997) The impact of focal dystonia on the working life of musicians. Performing Arts Medicine News; BAPAM. Proceedingsof the International Conference.GI. 16-GI. 24. Butler A. G, Duffey P. O.F. Hawthorne MA, Barnes M. P. (1998) The socioeconomic implications of dystonia. In: Fahn S, Marsden C. D and DeLong MR. eds., Advances in Neurology, Vol. 78 : Dystonia 3. Philadelphia: Lippincott-Raven. 349-358.

Butler A. G, Hawthome M. P, Duffey ROT, Gudex C.M. (1995) A comparison using a number of different rating scales measuring the effectiveness of Botulinum Toxin therapy in the treatment of dystonia and secondary dystonic spasms. MovementDisorders; 10 : 398. Duffey P. 0.17, Butler A. G, Hawthorne M. R, Barnes M. P. (1998) The epidemiology of primary dystonia in the north of England. In: Fahn S. Marsden C.D. and DeLong M. R. eds. Advances in Neurology, VoL 78: Dystonia 3. PHadelphia: Lippincott-Raven. 121-126. Duffey ROY, Butler A. G, Hawthorne M. F, Barries M. P. (1999) The prevalence and spectrum of primary dystonia in an English town. Submitted for publication to BMJ in 1999. Yet to be peer reviewed.

vii

Dystonia ý-A comprehensiveand longitudinal study in the North East of England

Medd D. Y. (1996) Counselling for dystonia patients. Part 1. TheEystonia Society Newsletter.; 24 ; 5-6. London. TDS. Medd D. Y. (1997) Counselling for dystonia patients. Part 2. The Dystonia Society Newsletter. ; 25 ; 5-6. London. TDS. Medd D. Y. (1997) Dystonia and hypnosis. ContemporatyHypnosis: Vol 14,No 2, 121-125. Medd D. Y. (1999) Clinical Report :A single-casestudy of generalised dystonia and hypnosis, with unexpected immobility and an untoward effect. ContemporaryHypnosis: Vol 16, No 1,45-48. Medd D. Y. (1999) Hypnosis with selected movement disorders. Contemporary Hypnosis: Vol 16, No 2,81-86. Gudex C.M, Hawthorne MR, Butler A. G, Duffey P.O.F. (1995) A cost-utility analysis of Botulinum Toxin therapy in the treatment of dystonia. MovementDisorders: 10 ;3 73. Gudex C.M, Hawthorne M. R, Butler A. G, Duffey P.O.F. (1997) Measuring patient benefit from Botulinum Toxin in the treatment of dystonia : Feasibility of Cost-Utility Analysis. PhannacoEconomics: 12: 6; 675-684. Gudex C.M, Hawthome,MR, Butler A. G, Duffey P.O.F. (1998) Effect of dystonia and Botulinum Toxin treatment on health-related Quality of Life. Movement Disorders: 13 :6; 941-946. . Whitaker J, Butler A. G, BarnesM. P. (1998) Botulinum Toxin treatment for people with dystonia by an outreach nurse practitioner -a comparative study. Report to the Northern and Yorkshire RegionalHealth Authority (NYRHA). Whitaker J, Butler A. G, Semlyen J.K, Barnes M. P. (1999) Botulinum Toxin treatment for people with dystonia treated by an outreach nurse practitioner -a comparative study. Submitted to the Archives of Physical Medicine and Rehabilitation in 1999. Currently under peer review.

Note : All of the enclosed is my own work except where specifically noted. Although a number of the above publications are jointly authored, these have been specifically noted when abstractshavebeenquoted in the text. A. G.Butler, Esq. I st September 1999

Vill

TABLES AND CHARTS Chapter 2: Background CHART I:

Page

Schematicshowing the different researchprojects under-taken

Chapter 4: Classification TABLE2: TABLE3: TABLE4: TABLE5: TABLE6: TABLE7:

Classification of Dystonia New Actiological Classification Gene Nomenclature for the Dystonýias Nomenclature of the Focal Dystonias The Cervical Dystonias The Segmental Dystonias

9 10

12 1

Chapter 5: Previous Epidemiologies TABLES: TABLE9: TABLE 10:

Distribution of patientsat Dystonia Clinical Research Center in New York from 01.09.73to 3 1.03.86 Distribution of patientsat Dystonia Clinical Research Centre basedon Onsetand Type Categories Prevalenceof the various forms of dystoniain the North East region basedon the Mayo clinic estimates

16 17 17

Chapter 6: Treatments TABLE II:

Injection Sites for Spasmodic Torticollis

26

Chapter 8: Data Collection and Instruments CHART 12 : Sourcesusedin correctly identifying and diagnosingeach subject

36

Chapter. 9 : The Cost Utility Analysis TABLE 13 :

Sequence of CUA questionnaires used during injection cycles

37

Chapter 16 : Diagnoses, previous and current TABLE 14:

Onset vs Diagnosis in each year

ix

53

Chapter 17 : Treatments, previous and current TABLE TABLE TABLE TABLE TABLE

15: 16: 17: 18: 19:

TABLE20: TABLE21: TABLE22: TABLE23:

Different treatmentsused andvarious effects recorded Measurementof Botulinum Toxin Usage Different side effects Length of time affectedby all treatments Different specialistsconsultedprior to diagnosis and alternativetreatmentsgiven Different Primary Medicationsgiven Different SecondaryMedicationsgiven Frequencyof Prescription Different Methods of Prescription

56 57 58 59 60 61 62 63 63

Chapter 18 : Comorbidity and The Quality of Life TABLE24: TABLE25: TABLE26: TABLE27: TABLE28: TABLE29: TABLE30: TABLE31: TABLE32: TABLE33:

Comorbidity of patients- directly relatedto dystonia Comorbidity of patients- indirectly related to dystonia. Comorbidity of patients- not directly related to dystonia Other Major Illnesses Types of Different Movements Severity of Movement Degree of control over musclespasms Degree of control over involuntary movements/ abnormalpostures Where is the greatestpain ? Length of time of SpontaneousRemission

64 65 65 66 67 67 68 68 69 70

Chapter 19 : Genetics TABLE 34: TABLE35:

Relationshipto others affected Type of spasmreported in the allegedlyaffectedpeople

71 72

Chapter 20 : Social Implications TABLE36: TABLE37: TABLE38: TABLE39: TABLE40: TABLE41: TABLE42: TABLE43: TABLE44: TABLE45:

Social and Economic Group (SEG) Categories Social and Economic Groupings (1996) Social and Economic Groupings (1998) Comparison of Pain and Discomfort to Anxiety and Depression levels on EuroQol vs SF36 Number of children of the patients Time off work taken to obtain treatment in previous 4 weeks Days off work for patients and partners Distance travelled to hospital(s) Travel time to old and new hospitals Method of travel to old and hospitals

x

73 74 75 76 78 78 79 79 80 80

Chapter 20 : Social Implications (cont) TABLE 46: TABLE 47: TABLE 48:

Type of major event and what they think causedI. T.D. With whom do you live In what type of accommodation

81 82 82

Chapter 21 : Economic Implications TABLE49: TABLE50: TABLE 51: TABLE52: TABLE53: TABLE54:

Employment Status Socio-EconomicGroups Comparisonof Highest Patient Statusto the GeneralPopulation Criteria used for inflation Chi-SquareTests: Comparisonsin Income over time Different benefitsreceived

83 84 84 85 85 87

Chapter 22 : Psychological Profiles TABLE55: TABLE56: TABLE57: TABLE58: TABLE59: TABLE60: TABLE 61: TABLE62: TABLE 63: TABLE64: TABLE65: TABLE66: TABLE67: TABLE68: TABLE69: TABLE70:

Social Scores Physical Scores Self-Care Scores Leisure Scores Other Scores Disabling / Uncomfortable / Disfigurement Speed / Strength Scales , Postural / Movement Scale Evaluative / Aesthetic Scale Tension Scale Male / Female Scale The Beck Depression Inventory Self-Esteem Scores The Impact of Dystonia Primary Carer's Questionnaire Personal Statements

Chapter 23 : Environmental TABLE71: TABLE72: TABLE 73: TABLE74: TABLE75: TABLE76: TABLE77: TABLE78: TABLE79:

89 89 90 90 90 91 91 92 93 93 93 94 95 96 97 98

Profiles

Which arm or handis affected Self-reportedThyroid Gland activity What triggered your dystonia? How light affected 138 people SpasmTest Results Different Allergies Food and Drink affected Number of cups of tea and coffee drunk per day Types of material and degreeof affectedness xi

99 100 100 101 102 103 104 105 106- 108

Chapter 23 : Environmental TABLE 80 TABLE 81

Profiles (cont)

from the subjectson the conductof this Responses researchproject Responses reactingto outsidestimuli

109 110

Chapter 24 : The Dystonia Society TABLE82: TABLE83: TABLE84: TABLE85: TABLE86:

Membershipof TDS When did peoplejoin TDS No of membersof TDS The Welfare Benefits ServiceUsage Resultsof Welfare Benefit Advice

112 113 113 115 115

Chapter 25 : Spasmodic Torticollis TABLE 87: TABLE 88:

What is the position of your head? What is the direction to which your headmoves ?

116 116

Chapter 26 : The Outreach Nurse Practitioner Project (ONPP) TABLE89: CHART 90: CHART 91: TABLE92: TABLE 93: TABLE94: TABLE 95: TABLE96: TABLE97: TABLE98: TABLE99: TABLE100: TABLE101: TABLE 102: TABLE 103: TABLE 104: TABLE 105: TABLE106: TABLE107: TABLE 108: TABLE109: TABLE109:

Numbers participating in the research project The Home Group The Clinic Group No. of people completing each Questionnaire No. of subjects and length of time to completion Geographical Location of patients in ONIPP. TDS Membership as of May 1997 The SEG of both groups Marital Status of the two groups No. of injectors during the study A comparison over time No. of injections hurting Comparison of injector's knowledge and attitude Comments on the service received Patients' own knowledge of their own dystonia What makes the spasms worse ? What makes the spasms better ? Treatment and service compared to a year ago Patients' comments about the whole project Preference for home or the clinic (a) Final comments on the ONP project from the Home Group (b) Final comments on the ONP project from the Clinic Group

.Xii

119 120 121 122 122 123 124 125 126 129 130 131 133 134 135 136 137 137 138 138 139 139

Chapter 28 : Epidemiology TABLE I 10 The GeographicalDistribution of Dystonia within the ESD The spreadof dystoniaspasmsin the region TABLE III TABLE 112: The spreadof dystoniaspasmsin the rest of the ESD CHART 113 :A Map of the North of England showingthe dystonia of prevalence

144 145 146 147

Chapter 33 : Social and Economic Implications TABLE 114: Vicious Circle Syndrome- The Theory TABLE 115 Vicious Circle Syndrome- The Evidence TABLE 116 Interventionsthat break the Circle !

xill

158 159 160

Dystonia -A comprehensiveand longitudinal study in the North East of England

ABSTRACT Dystonia is a little known neurological diseaseof the central nervous system and involuntary by disorders, of a group of related movement characterised and consists it is Although spasms of muscle contraction. nearly 90 years since this prolonged been disorder first little had was named, relatively research undertaken neurological into dystonia,for the first 65 years and it was not until the mid 1970's that researchers look disorder. to the at started This particular programmeof researchhas taken place exactly over a six year period, in large different This May 1993, to study. areas of and relates a number of starting is it is has dystonia far thought, that than proven more prevalent previously research next to Parkinson's Disease in degree of prevalenceand is far more common than it known Disease, better Motor Neurone and conditions, yet neurological suchas other largely unknown to most membersof the medical profession and the general remains public at large. Dystonia has been historically extremely difficult to diagnosis and this meant it has been previously very difficult to obtain sufficient numbersfor study, which in turn has created a number of significant social and economic consequences,which has mainly meant that most cases of people with dystonia have remained undiagnosed or misdiagnosedfor manyyears. This researchwas designedto measurethe severity and prevalenceof dystonia in the life had implication disease has UK, the the the the on working and part of northern environmentof each patient and how that person is coping with the various personal, deterioration by family the gradual social and onset and potential relationshipscaused during life disorder, the the a number of each patient of as well as measuring quality of of different therapies. Although there has been research into other neurological disabilities, very little is known about the implications that dystonia can have on the affected person and their families. This is the first time that all types of dystonia have been studied and that certain related subjectshavebeenspecificallyincluded. This researchhas been enormouslyhelped by the tremendousexpansionin the use of Botulinurn Toxin therapy and although an enormous amount of work has been completed and accomplished during this research programme, it should never be forgotten that the subjectsof this thesis are real people and that the implications and results of this researchhave had, and will have, a tremendousimpact on their lives and that of their families.

xiv

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART ONE: INTRODUCTION CHAPTERI Introduction

Aim and of the Research

What is Dystonia ? Dystonia is a previously little known neurological disease of the central nervous Z: ) (CNS) and consistsof a group of related movementdisorders, characterisedby system involuntary and prolonged spasms of muscle contraction. It has been classically defined as "a syndromeof mistainedmuscle contractions,frequently causing twisting and repetitive movements,or abnonnalpostures. " (Fahn et a], 1987) It can affect the whole body or a single part of the body or some combination between the two. Although it is nearly 90 years sincethis neurological disorder was first named (Oppenheim,1911), relatively little researchhad beenundertakeninto dystonia for the first 65 years,apart from the odd periodical review (Herz, 1944). Since the 1970's, a number of eminent medical people have started to researchinto dystonia and this has increased its 'popularity' amongst medical researchers. However, to date, there hasnot beena comprehensivelongitudinal study of the disease within a specific population anywherein the world.

What is the aim of this research? This researchis designedto measurethe severity and prevalenceof dystonia in part of the LJK, the implication the diseasehas had on the working life and environment of each patient and how that personis coping with the various personal, social and family relationshipscausedby the onset and potential gradual deterioration of the disorder, as well as measuringthe quality of life of eachpatient in a number of different therapies. Although there has been researchinto the prevalenceof most neurological disabilities (Hewer, 1993), very little is known about the implications that dystonia can have on the affected person and their families, apart from limited circulation articles in The Dystonia Society (TDS) newsletters(Butler, 1995). The purpose of this research was designed to measurethe quality of life of each patient, the severity and prevalenceof dystonia in the UK, the implication the disease has had on the working life and envirom-nentof each patient and how the patient is coping with the various personal, social and family relationships causedby the onset and potential gradual deterioration of the disorder.

Dystonia -A comprehensiveand longitudinal study in the North East of England

in dystonia terms of reduced mobility, that causes known considerable morbidity It is difficulties interaction low embarrassment, poor social and with self-confidence, pain, (Marsden and Quinn, 1990) but this will be the first time that all aspects employment disorder has been the in life comprehensivelystudied. with people of

Why was it necessaEy2 During private correspondencein July 1991 between the author and the late David Marsden, Professor of Neurology at the Institute of Neurology in London, David Marsdenstatedthat " iio otie Imowshoiv matiy casesof dystoWaexist ih the U.K. " When The Dystonia Society was first established,basedon Marsden's patients' list in 1983,there were only 6 known dystonia patients in the North East, all of whom were Generalisedand most of whom had Familial (ie, geneticallyinherited) Dystonia. This is not to say that there were not other people correctly diagnosedat the time in the North East, but it does indicate that they were not known outside of their own medical centre nor were they on any formal (or even informal) epidemiological register. When the first Self-Help Group (SHG) was establishedin the North East in 1990, there were 40 known people with dystonia on the initial mailing list, including 25 TDS members. In a survey of 705 people with dystonia, it had been establishedthat one third of all dystonia.suffererstake 5 years or more to diagnose;37% said that at least one doctor had suggestedthat their condition was "all in the mind' and 32% had beenreferred at one time to a psychiatrist; 66.7% of all sufferersneededat least 5 consultationsbefore diagnosisand 65.7% were misdiagnosedat somestage(TDS, 1993). At the time of the eventualstart of this research,TDS (NE) had only 56 members,with only a further 87 people with dystonia known in the region. This meant that the researchstarted officially on 6th May 1993 with only 143 known people displaying at least one of the various symptomsknown as dystonia in the geographicalarea known as the North East of England. Moreover as the author was not medically qualified, it was important for a detailed literature review and a limited training programmeto be carried out prior to the start of this research. This took over two yearsof intensivestudy from 1991 until 1993 and included attending a number of researchand training seminarsthroughout the U.K., someof which are noted throughout the following pages. Finally, it was necessaryas the author felt that the numberof people with dystonia had always been under-estimatedand therefore someform of in-depth investigation had to be carried out before others could categoricallystatethe true relationshipsinvolved.

1)

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART ONE: INTRODUCTION CHAPTER 2 Background How did this researchcome about ? " One of the main problems about dystonia in this country is that we have no really how idea common it is. It is likely that the majority of cases are about good by the medical services and so research into the social and economic undetected aspectsof the caseswe know about would not really reflect what is actually going on in thepopulation. Infact, one of the things we really do need to know is how common dystonia is, and this is difficult to do without actually doing a door to door survey, do I not think is the sort of thingyou have in mind." which The above is an extract from a letter to the author by Dr Nick Fletcher, at the time Senior Registrar in Neurology at St Bartholomew's Hospital in London and the Medical Advisor to the Dystonia Society, dated 10th August 1992. This was the initial stimulus which started the author on a task which has been far longer than at first envisagedand at times very difficult to accomplish,but it has since become "one of the most important pieces of researchinto dystonia, outside ofpurely medical research tofind a cure " (Source : private correspondencewith the Dystonia Medical ResearchFoundation in the USA).

Why was it done in the wgy it was One of the main reasonswhy there has been a problem in the diagnosisof dystonia is mainly historical, as it was not even classified as a neurological disorder until quite recently. "Until the' 1970's mostpatients with dystonia were referred to psychiatrists in the belief that these curious motor disorders were an expression of an unhappy mind. Vigorous efforts over thepast two decadeshave now, however,establishedthat the various syndromesof dystonia are the result of abnormal brain fiInction, usually in the basal ganglia. " (Marsdenand Quinn, 1990). It was therefore necessaryto firstly find out how many people had dystonia in order to obtain sufficient numbersof subjectsfrom which to draw the necessaryinformation on which to researchfor this project. Access to people who have dystonia was consideredto be the primary difficulty in carrying out any sort of dystonic researchproject (Butler et al, 1998) and as the very processof identifying those peoplewith dystoniameant first having to obtain a correct diagnosis, the need was seen to start by firstly carrying out a comprehensive epidemiologyof dystonia.

3

Dystonia -A comprehensiveand longitudinal study in the North East of England

When did it start ? The researchstarted officially on 6th May 1993 and continued full time until 5th May 1999 for an inclusive period of 6 years exactly. However the reader should be aware that although the entire processwas continuousthere were, of necessity,a numberof different researchprojects, which becamepart of the entire process,as follows :Oct 1996 - Mar 1997 May 1993- June 1994 Aug 1995 - July 1997 May 1993 - Sept 1996 May 1993 - Sept 1996 May 1993 -Dec 1997 May 1993 - May 1999

Dystonia affecting Working Musicians A Cost Utility Analysisof Botulinum Toxin The Dystonia Nurse PractitionerProject The Epidemiology of Primary Dystonia The Psycho-SocialResearchinto Dystonia The Socio-EconomicResearchinto Dystonia The Epiden-dologicalSurvey of Dystonia .

= 0.5 years = 1.2 years = 2.0 years = 3.4 years = 3.4 years = 4.7 years = 6.0 years

Although each part of the above researchis free standing as such, the whole was always designedto form the author's overall researchfor his Doctor of Philosophy degreethesis. The following chart showsthe relationsl-ýpbetweenthe various projects and the number of patientsinvolved in eachas well as the length of time taken. CHART No 1. Schematic showing the different research projects undertaken. Dystonia A comprehensive and longitudinal study of the implications of dystonia within the population of the North East of England

The Epidemiological Survey of Dystonia 6th May 1993 - 5th May 1999 All data available from 937 subjects

Cost Utility Analyis

of Botulinum

Toxin

The Epidemiology of Primary Dystonia May 1993 - September 1996 372 subjects living in the North East I

May 1993 - June 1994 199 patients + 31 controls = 230 subjects

Dystonia Nurse Practitioner Project August 1995 - July 1997 126 selected cases

Dystonia

Psycho-Social Research into Dystonia May 1993 - September 1996 Data from 346 subjects I

T

--Socio-Economic Research into Dystonia May 1993 - December 1997 Data from 243 subjects

Working Musicians affecting October 1996 - March 1997 3 selected case histories

There have beena number of publications,madeas a direct result of the researchdone by the researcherand his fellow workers, which are all listed on pagesvii and viii.

4

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART TWO:

DYSTONIA

CHAPTER 3 Literature

Review

GeneralReview Before 1976, dystonia.was often considered to be a pyschogenic / psychological/ disorder and therefore,as it was a vital part of the initial literature review to psychiatric define wl-kh classification system should be used in the researchprogramme, it was necessaryto examinewhat alreadyexisted. Intemational Classificationof Diseases The International Classification of Impairments, Disabilities and Handicaps (ICIDH) had been developed by the World Health Organisation (WHO) as a way of interrelating diverse data on individuals to illuminate the impact of disease,to promote a more sensitiveuse of terminology and exposeweaknessesin thinking about disability. There has been some criticism of the WHO definitions in saying that " they pay insufficient attention to the external deterininatioti of hatidicap and are yet another meansof labelling or stigmatising the handicapped" (Wood, 1987). It also shouldbe remembered that ICIDH is not a classification of persons but of the types of experiencesto which they may be exposed. " It is not a research or assessmenttool but a meansof organising data in a useful ivay. It ivas never designedas a basisfor orgatfising people into categories" (Wood et al, 1989). Further work was therefore neededto assessICIDH's usefulnessto these particular dystonia researchprogrammes.Eventually all ICEDH codes were rejected, including ICD8 and ICD9 (as used in the NHS prior to the implementation of ICDIO in April 1995). The reasonsare obvious once one looks, for example,at the classificationof dystonia in ICD9 (NHS Man. Exec, 1978). Code 333.6 shows Idiopathic Torsimi Dystonia, with only Dystonia Musculorum Deformans or (Schwalbe) Ziehen - Oppenheim Diseaseas a sub category. Code 33.3 3.7 shows Symptomatic Torsioti Dystonia, with only Athetoid Cerebral Palsy or Vogt's Disease and Double Athetosis as sub categories. And finally, all the focal dystonias are shown under code 333.8 as Fragmetits of Torsioi7Dystoifia, with only Blepharospasm,Qrganic Writer's Cramp, Orofacial Dyskinesiaand SpasmodicTorticollis shown as the sub categories. Any person, who knows even the slightest amount about dystonia and its various subcategories,will know that the abovecategoriesdo not reflect either the current known different stages of dystonia nor the most up-to-date classifications of dystonia. Therefore it becamequite obvious quite quickly that some other systemas opposedto that currently in use within the NHS had to be usedto classify dystonia.

5

Dystonia -A comprehensiveand longitudinal study in the North East of England

The Read Codes The author then studied and eventuallyrejectedthe new Read Codes which are being , gradually implementedin the NHS, as they merely follow the ICD model (NHS Man. Exec, 1993). For example;the three ICD9 categoriesshown above equal the Read Code categories of F136, F137 and F138. Even SpasmodicDysphonia is still being categorisedas R0444, i. e., under 'Symptoms, Signs and III-Defined Conditions'. The reasonfor all these rejections was on the grounds that they were either too generalor for detailed enough a proper dystonic classification. not Epidenýological Surveys of Dystonia

Any epidemiological survey of dystonia (ESD) is normally, by deflnition, quantitative in nature as its purposeis initially merely to identify the actual numbersof people with dystonia within a given area. However, as has been seen by earlier reference to Professor David Marsden and Dr Nick Fletcher, accessto people who have dystonia was considered to be the primary difficulty in carrying out any sort of research programme and as the very process of identifying those people with dystonia meant first obtaining a correct diagnosis,the opportunity aroseof having previously unknown (unidentified and undiagnosed)patientsavailablefor other dystonia researchprojects. As can be proved all the research projects stem from the initial need to correctly identify sufficient numbers of people with dystonia. Therefore, in practice, the epiderniolo, gy was the tool which identified the subjectswith dystonia and although it was also a piece of important researchwithin itself, it led to the different and varied researchprojects and in particular the data for this researchthesis. However, in order to carry out an epidemiologyof dystonia, a review of the literature on the subject revealedseveralimportant areasin both the design and implementation of such a study. According to one of the world's foremost neuroepidemiologists: " Neuroepidemiologymay be defined as the study of the distribution and dynamicsof neurological diseases in human populations and the factors that affect those characteristics. " (Schoenberg1978) It is precisely for these reasons that this ESD is of such importance because it attempts, as never before, to identify all case of (dystonia) in a ivell-define (author's emphasis). Other studies (Nutt et al, 1988 and Nakashima, pooilation 1995) have been exclusivelyclinic basedand therefore will have a definite referral bias and do not carry the samevalidity as one that attempts 100% identification. Those epidemiologicalstudiesthat were not clinic based(Alter, 1976; Korczyn, 1980;Zilber, 1984 and Li, 1985) are flawed in that either the ascertainmentof their diagnosiswas incomplete or the nature of their criteria was limited in that they were not purely designedto just pick up dystoniaand therefore are not as accurateas the data from the North East study.

6

Dystonia -A comprehensiveand longitudinal study in the North East of England

Disability in General Five separate,but linked, surveysinto disability in Great Britain have beenpublishedas six separatereports (No 1: Martin et al, 1988; No 2: Martin and White, 1988; No 3: Bone and Meltzer, 1989; No 4: Martin et al, 1989;No 5: Smyth and Robus, 1989;No 6: Meltzer et al, 1989). The surveyswere undertakenfrom 1984 to 1988 and are the most comprehensiveand recent availablefrom the Office of Population, Censusesand Surveys(OPCS). Their aim was to find out the number of disabled people and the severity of their disability and they estimatedthat there were 6,560,000 people with disabilitiesin Great Britain in 1985, although it has proved impossibleto accurately differentiate people with any form of dystordcspasmwithin this estimatefrom the data. The types of disability and severity were distinguishedaccording to ICIDH, a feature of the survey being the construction of the measureof severity of disability from I (low) to 10 (high). Of the six and a half million disabledpeople in the UK with at least one disability, only 400,000 (7%) were living in communalhomes, and this numberis related in direct proportion to the severity of the disability. These reports contain a lot of very detailedfindings but it has proved difficult to relate any of them to dystoniain particular or evenother neurological movementdisordersin general. However, one particular finding will and can be comparedin this researchie, the fact that more women are affectedthan men. Approximately one in every nine people in the U.K. is disabledin one form or another and one family in every six has a memberwho has a disability, neverthelessthe OPCS reports define disability as medical criteria whereas WHO (ICEDH) classification of disability covers a range of medical conditions which may not be handicapping depending on the individual's ability to overcome the socio-economic and environmental obstacles (OBrien and McFetridge. 1991). The views of disabled people in generalon disability show that the use of terms like 'the disabled' to describe 'physically handicapped people' is often deplored and attempts by the medical profession to understandhow disability affects a person'slife are describedin at least one journal as "inadequate" and "likely to promote stereotypical views" (Brisenden 1986). Other researchhas shown that the discriminationfaced by disabledpeople in a society of 'non-nal'people acts as a catalyst to the perpetuation of passivity and dependence amongst people with disabilities. A lack of facilities, and inadequatedesigns,ensure that they constantly face problems ranging from gaining accessto a building-to facing constant medical interventions,regardlessof their value (Barton 1989). One needsto discover methodsby which disabledpeople can achievegreater independenceand an equal place in society, exercisingfreedom of choice for independentliving as a right, rather than a privilege (Brisenden 1986 and Oliver 1990). Whilst researchingthe field of disability, it became quickly evident that there were several schools of thought in in dystonic disability the the general, population particular, and movement within about the different theoriesregardingdisability.

7

Dystonia -A comprehensiveand longitudinal study in the North East of England

The Social Model of Disability. One of these 'schools of thought' relatesto the "social model of disabifitV%which is a vast potential area of researchin itself, well beyond the brief of this researchthesis, it nevertheless should be mentionedand explained. In essence,this view of disability " disability has been seen as the problem of the individual traditionally, that states and it has been the individual who has had to change or be changed by professionals through rehabilitation or cure " (Michael Oliver, Professor of Disability Studiesat the University of Greenwich, spokenat a seminaron disability in Newcastle-upon-Tyneon 3rd February 1994). Furthermore, according to Professor Oliver, " Now, disabled people and their have described, from their own experience, how it is economic and organisations social barriers which stop people with impairments participating fidly in society. Thesebarriers are so widespreadthat we are preventedfrom ensuring a good quality of lifefor ourselves" (Oliver, 1990). This explanation is known as the 'social model of disability, becauseit focuses on society'sdisablingenvironmentsand barriers of attitude, rather than on individuals with impairments. The social model was formulated by disabled people and now also has been acceptedby a number of non-disabledacademicsand professionals. It stresses humanrights and the equality of opportunities. Nlike Oliver has suggestedthat " motley spent oil medical research into curesfor disabling illnesses and conditions is wasted motley and most funds raised for this purpose are allocated and administered by able-bodied people, and the obsessive pursuit of a cure can ruin as many lives as the illness or condition itsetf " (Oliver 1990) and at the Newcastle Seminar,he further assertedthat " disabledpeople do not want to be cured and such (research)junds would be better utilised it] removing some of the social barriers which limit thepotential of disabledpeople." Oliver predicts that " the disability movementwill contimie to confront the disablism in service provision and professional practice, but the tactics of confrontation are beginning to change. In place of writing books and articles and speaking at conferences,the 1990's may well becomethe decade of direct action on disability. 777isdirect action will build on thefailures of the past, challenge the vestedinterests of thepresent andforce a restnicturing ofstate weýfarethat is sojar-reaching that we may not evenrecognise the weffiarestate in the twenty-first century." (Oliver, 1991) The author's personal experienceis not compatible with this view of disability and conversationsof an anecdotalnature, since February 1994, have suggestedthat most people with dystonia (which, for those with primary dystonia at least, is potentially a curable disease)want a cure, if one can be found - although most agree that more could be done by way of practical help now !

8

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART TWO: DYSTONIA CHAPTER 4 Classification Up until 1996, it was necessaryto use the Ad Hoc Committee of the Dystonia Medical ResearchFoundation criteria (Fahn, 1988)which classifiesdystonia in three ways. TABLE 2. Classification of Dystonia

By cause

(II)

(a) Idiopathic (b) Symptomatic

(Primary Dystonia) (SecondaryDystonia)

By age at onset (a) Childhood-onset : 0-12 years (b) Adolescent-onset: 13-20 years (c) Adult-onset : >20 years

(III) By distribution

(a) Focal body single the only of affects a part (b) Segmental - affectstwo or more connectingparts (c) Hemidystonia,- affects an ipsilateral arm and leg only (d) Multifocal two affects or more non-contiguousparts (e) Generalised - definedas segmental,plus at least one leg.

There were generally considered two causes of dystonia; idiopathic (familial and sporadic) generally with an unknown cause or symptomatic with a known (or assumed)cause. There are many known causesfor secondarydystonia which have beenfisted within the medicaltextbooks. The age at onset classificationis only normally used where it can give a fairly accurate measureof the distribution of the disorder over time. A rough rule of thumb guide is the earlier one gets this disorder the greater the chanceof it, spreadingthroughout the entire body. However, the opposite is also true, in that the later one developsa form of dystonia,the more locafised(or focal) it remains. The final classificationis the position which the disorder currently occupies within the humanbody. Theseare generaRydescribedas above,although there are also a number ' of sub-divisionswithin thesecategories. It should be rememberedthat the diseasewill often move throughout the human body over time and therefore one can often see a young child developing dystonia in a limb, which will progress until it is present in most (if not all) parts of the entire body.

9

Dystonia -A comprehensiveand longitudinal study in the North East of England

It is therefore often assumedthat once one developsthe disorder it is with one for the rest of ones life. This is generallytrue, but there have been a number of exceptionsto this and indeed a number of the present cohort of patients have gone into remission, somefor many years. Each of these classificationswill be discussedin greater detail later, but it must be realised from the start that the classification with regard to 'age by onset' and by 'distribution' hasremainedconstantthroughout this entire time. Aetiology The new aetiological classificationhas really come about as a direct result of our everchangingand improving understandingof this disorder. As a result of a paper (Fahn et al, 1998) presentedin Marni, Florida at the 3rd International Dystonia Symposiumin October 1996, the aetiological classification of dystonia has greatly changed. However, it should be realisedthat this has not changedeither its 'age by onset" nor essentiallyits 'distribution', only its aetiology. Thesemain changesrelate to the recent discoveriesin the geneticsof dystonia, which indicate that more than gene can cause idiopathic dystonia. This major breakthrough owes much of its reasoningto the recent classification of parkinsonism,where it has proved useful to divide " parkinson disorders into primaty (usually called Parkinsons Disease), secondary (due to environmental or structural causes),Parkinson-plusspidromes (in which other neurologicfeatures in addition to parkinsonism are also present) and heredodegenerative Sp1dromes (such as Hallervorden-Spatz disease, Wilson's diseaseand Huntington's disease), which can bepresent with aparkinsonian picture " (Fahn, 1995). The idea is to divide dystonia into four main aetiological classification, each one relating to specific other " recognisedentities that appear to be distinct and each may be enlarged in thefuture asfiirther genetic, pathological and biochemical advances are made" (Fahn et al, 1998). TABLE 3: New Aetiological Classification

I: Primary Dystonia

syndromeswith dystonia.as the sole phenotype

2: Dystonia-Plus

distinct from the following two types and includes dystonia with parkinsonism, dopa-responsive dystonia, dopamine-agonist responsive dystonia, with myoclonic jerks and dystonia-myoclonus

3: SecondaryDystonia.

non-genetic,developsdue to enviromnentalfactors

4: HeredodegenerativeDystonias neurodegenerationswhich produce dystoniaas a

prominent feature, e.g. Huntington's, Wilson's, etc

10

Dystonia -A comprehensiveand longitudinal study in the North East of England

Whereasthe previous aetiological classificationof dystonia was essentiallydivided into two, ie primary (idiopathic) and secondary(symptomatic), recent discoveriesinto the geneticsof dystonia have meantthat whHstthe original three categoriesfor describing patients, namely age at onset, distribution and aetiology remain, the aetiological category can be expandedto include four sub-categoriesas shown in Table 3 above, which the genesbeing defined more specificallyin Table 4 below. The gene nomenclatureshave been expandedrapidly and the acceleration has greatly increased over the past year and at the recent National Spasmodic Torticollis Conferencein October, 1998 in San Diego, USA, the following geneswere identified and discussed. TABLE 4: Gene Nomenclature for the Dystonias

DYTI Gene: 9q34.1, Auto-dominant, early-onsetand limb onset DYT2 Gene: autosomnal-recessivedystonia DYT3 Gene: Xq 13, Lubag DYT4 Gene: a whispering dysphoniafamily DYT5 Gene: 14q22,dopa-responsivedystonia,GTP cyclohydrolaseI gene DYT6 Gene: 8p21-q22, mixed type dystonia DYT7 Gene: 18p, familial tor-ticollis DYT8 Gene: 2q, PNKC, paroxysmal DYT9 Gene: Ip, CSE, paroxysmal DYT 10 Gene: not imp., PNKC, paroxysmal DYT II Gene: myoclonic, Auto-dominant DYT 12 Gene: l9q, Rapid OnsetDystonia Parkinsonism,reduced penetrationand Auto-dominant

These will not be discussedany further at this stage, but essentiallythey are placed hereto show that geneticsare beginningto play a more and more important part in the evaluationof dystonia. Distfibution The distribution of the dystonias,as describedin Table 2, has not changedbut these need to be describedin greater detail as one of the reasonswhy dystonia is so poorly acknowledgedis often due to the reasonthat someonewith a focal dystonia does not know they have a neurological condition, even though they might know the given nameof what they have. The reasonfor this is often due to the various namesgiven to, in particular, the focal dystonias. The following table shows the distribution of the dystoniasas well as the namesgiven to thesevarious conditions, which goes to explain why these different conditions are often misdiagnosedor more often not thought to be related, outside of medicalcircles.

II

Dystonia -A comprchcnsivcand longitudinal stud), in the North East of England

TABLE 5. Nomenclature of the Focal Dystonias.

Blepharospasm

(or the affecting eyes specificallythe eyelids) -

Dystonia Dysphagia

- affecting swallowing Focal Dystorýa of a Limb - affecting an arm, a leg or a foot (a PeripheralDystonia) Lingual Dystonia

the tongue affecting OromandibularDystonia -affecting thejaw and mouth

SpasmodicDysphonia

larynx the affecting and speech -

SpasmodicTorticollis

the affecting neck Unilateral Blepharospasm- affecting one eye (or one set of eyelids) only Writer's Cramp hand fingers (a Peripheral Dystonia) the affecting and -

Hemi-facial Spasmis a 'dystonic type' spasmaffecting one side of the face only. It has been argued that all casesof Herni-facial Spasm are not classified as being a focal dystonia (Elston, 1997). The results of this research,to be discussedlater, could argue that this is a false assumptionand that a number of so-called 'herni-facial spasms'are in fact a focal dystonia of one side of the face, or in particular one set of eyelids,but in order to satisfy current medical opinion the term 'Unilateral Blepharospasm'hasbeen included aboveto differentiate betweenthe two definitions. Some of the above can be defined even more, for exampleCervical Dystonia (which is the technically correct ten-nfor what is generallyknown as SpasmodicTorticollis) can be defined in four distinct ways dependingon how the head moves or in what direction the headturns. TABLE 6. The Cervical Dystonias.

Torticollis

Where the headtums either left or right

Laterocollis

Where the headtilts either left or right

Retrocollis

Where the headleansbackwards

Antecollis

Where the headgoes forward, so that the chin touches the chest

Therefore where someoneis described as having SpasmodicTorticollis to the right, eg-, ST (R), it meanstheir headturns to the right. Thesedefinitions have beenused in the script, except that Laterocollis is not generallyused and a turn or a tilt is usually both just called SpasmodicTorticollis and they are not differentiated.

12

Dystonia -A comprehensiveand longitudinal study in the North East of England

The SegmentalDystonias have also a number of different terminologies to describe 0 forms different of the disorder as shown on the table below. Rememberthat various SegmentalDystonia was originally described(Table 2, Page 9 above) as a dystonia which affects two or more connectingparts. TABLE 7. The Segmental Dystonias.

Axial Dystonia

Affects the trunk and neck

Brachial Dystonia

Affects an ann + trunk, both arms +/- neck or trunk.

Crural Dystonia

Affects one leg and trunk, both legs +/- the trunk.

Cranial Dystonia

Affects two parts of the cranium +/- neck musculature.

Cranial Dystonia can be sub-dividedand is often given other names,eg., CraniocervicalDystonia

Affects a part of the headplus the neck musculature.

Meige's Disease

Combinationof Blepharospasm& Oromandibular

Brueghel's Syndrome

Combinationof Blepharospasm. & Oromandibular

The Herni-dystoniasaffect an ipsilateral arm and leg only, ie., on the sameside of the body Nfulti-focal Dystonia affects two or more non-contiguous parts, eg., a left arm and right leg or a right hand and the neck, etc, etc.

Another way of describingGeneralisedDystonia, which is defined as a combinationof SegmentalCrural Dystonia and any other segment,is to say at least one leg +/- the trunk and another segment,suchas the headand the neck or the eyesand a hand,etc.

1)

Dystonia -A comprehensiveand longitudinal studý,in the North East of England

PART TWO: DYSTONIA CHAPTER 5 Previous Epidemiologies General.Review As previously defined :" Nettroepidemiology may be defined as the siudy of the distribution and dynamics of neurological diseasesin human populations and the * factors that affect those characteristics " (Schoenberg,1978) and as firstly the author was not medically qualified and secondlyno one knew how many people had dystonia in the North East of England, it was necessaryto undertake some basic training in epidemiologyin order to start the work. One of the important areas of research in the literature review was to define the difference between prevalenceand incidence.Prevalenceand incidence are related to each other as prevalence approximately equals incidence multiplied by the average duration of the disease. This was the point at which the research should start, ie., how many people had dystonia within a given population, because" the two most important considerations for the neuroepidemiologist in the design of studies are the representativenessof the population selectedfor investigation and the accuracy of the diagnoses in that population (and) because of ..... limited access to neurologic expertise; some individuals with the diseaseof interest may never seekmedical care or may never be correctly diagnosed. To avoid this problem, the neuroepidemiologist attempts to identify all cases of a particular neurologic disease in a weXdefilned population. (Schoenberg,1986). By 1990,researchhad shown that there had only been a few epidemiologicalstudiesof dystonia ; in the USA (Eldridge, 1970 and Nutt et at, 1988), Israel (Korczyn et at, 1980 and Zilber et at, 1984), the Republic of China (Li et at, 1985) and Spain restricted to gypsies(Gimenez-Roldanet at, 1988). Even then these did not accurately reflect what was required, as " the two most important considerationsfor the neuro-epidemiologistin the design of studiesare the representativenessof thepopulation selectedfor investigation and the accuracy of the diagnoses in that population. " (Schoenberg,1986). Since then there have been a number of limited studiesundertakenin the USA (Risch et a], 1995), Assuit in Egypt (Kandil et a], 1994) and Tottori in Japan(Nakashimaet al, 1995). However, the only researchwhich was specifically related to a known and defined population which was sirnilar to that present in the North East of England was that carried out at the Mayo Clinic in Rochester,Minnesotain the USA (Nutt et al, 1988).

14

Dystonia -A comprehensiveand longitudinal study in the North East of England

Results of previous epidemiologies

The only epidemiological evidence available of estimated prevalence from an survey of dystonia(and other neurologicaldisorders)which took place epiden-ýiological in Rochester,Minnesota, USA at the Mayo Clinic, is taken from files collected from 1950to 1982,basedon an averagepopulation from 1960to 1979 ofjust over 100,000 697 where caseswere examined and 34 casesof idiopathic dystonia found people, (Nutt et al, 1988) including II casesof SpasmodicTorticollis (Claypool et al, 1995). This small samplewith very wide confidenceintervals (17.2 to 47.9 per 100,000 and 0.41 to 12.4 per 100,000 for focal and generaliseddystonia, respectively at the 95% interval) formed the basison which all future prevalencepredictions were based. Thus the prevalencefor both idiopathic and symptomatic dystonia was estimated at 391 per million, or " 20,000people in Britain basis that 45% of the calculated on ... all cases of generalised dystonia and 10% of cases of focal dystonia are symptomatic" (Marsden and Quinn, 1990), although Marsden himself was quoted in the BMJ article as sayingthat 20,000 may be a "substantial underestimate". The only epidemiologywhich had been carried out in the population within the North of England,up to relatively recent times and in anythinglike a similar disorder, was the epidemiology of Parkinson's Disease (PD) which had been carried out in Carlisle, Cumbria between 1955 and 1961. Here incidencehad been shown to be 12.1 new casesper 100,000 population / year with the prevalencerates as of January I st, 1961 to be 112.5 per 100,000. (Brewis et at, 1966). The averageduration of PD in Carlisle in the early 1960's was 9.3 years. It had been reported as long ago as 1984 that " there have, as yet, been no formal community-basedsurveys of torsion dystonia " but that Huntington's Disease-had a mortality for US Whites of 1.6 per 1,000,000per year (Kurtzke et a], 1984) and that Wilson's Diseasehad a prevalenceratio of 1.6 per 100,000and the incidencerate was 0.2 per 100,000 per year in the Icelandic population (Gudmundsson, 1969) and a world-wide prevalenceof 3 per 100,000 (Scheinberget al, 1984). To gauge an idea about the prevalenceof dystonia, a table showing the distribution pattern of patients with dystonia is reproducedbelow (Fahn et al, 1988). A total of 932 patientswith dystoniawere seenby the Movement Disorder Group in the 13 years between September 1,1973 and March 31,1986 at the Dystonia Clinical Research Centrelocated at Columbia-PresbyterianMedical Centre,New York City in the USA. NB : It should be noted now that over the 6 years of the epiden-dologyin the North East of England from 6th May 1993 to 5th May 1999, one will see a total of 937 people diagnosed, as opposed to the 932 shown above which took 13.6 years to generate and complete. Therefore it will be interesting to see the differences in a purely regional epidemiology in the North East of England as opposed to that of a large hospital in New York which takes referralsonly.

15

Dystonia -A comprehensiveand longitudinal study in the North East of England

TABLE 8: Distribution of Patients at Dystonia Clinical Research Centre in New York from 01.09.73 to 31.03.86. Category of Patient

Idiopathic Number

Idiopathic Symptomatic Percentage Number

Symptomatic Percentage

Childhood-onset Focal Segmental Multifocal Generalised Unilateral 5ub-Total

11 34 6 70 2 123

1.2 3.7 0.6 7.5 0.2 13.2

5 10 3 54 15 87

0.5 1.1 0.3 5.8 1.6 9.3

Adolescent-onset Focal Segmental Multifocal Generalised Unilateral Sub-Total

21 8 5 13 3 50

2.3 0.9 0.5 1.4 0.3 5.4

3 10 2 5 3 23

0.3 1.1 0.2 0.5 0.3 2.4

271 150 2 11 1 435

29.1 16.1 0.2 1.2 0.1 46.7

33 11 6 9 8 67

3.5 1.2 0.6 1.0 0.9 7.2

17

1.8

16 90 24

1.7 9.6 2.6

625

67*1

30j=

32.8

Adult-onset Focal Segmental Multifocal Generalised Unilateral Sub-Total dystonia _Paroxysmal Tardive dystonia Psychogenicdystonia TOTAL In the above chart :-

The categories of paroxysmal, tardive and psychogenic dystonia are shown removed from the idiopathic and symptomatic dystonias in order to list them as separateentities. 2) Their definitions were explainedin the original text and no distinction was madeas to age at onset for theselast three categories. Three patients with diurnal fluctuations are included among the childhood-onset idiopathic dystonia.

16

Dystonia -A comprehensiveand longitudinal study in the North East of England

TABLE 9: Distribution of patients at Dystonia Clinical Research Centre based on Onset and Type Categories. Onset Childhood Adolescence Adult Total

Idiopathic 123 50 435 608

Percentage 20.2% 8.2% 71.6% 100%

Symptomatic 87 23 67 177

Percentage 49.2% 13.0% 37.8% 100%

lype Generalised Multifocal Segmental Focal Hemi-dystonia Total

Idiopathic 94 13 192 303 6 608

Percentag 15.5% 2.1% 31.6% 49.8% 1.0% 100%

Symptomatic 68 11 31 41 26 177

Percentage 38.4% 6.2% 17.5% 23.2% 14.7% _100%

The North East of England (including Cumbria) The population of the five northern countiesof England, ie Cumbria, Northumberland, Tyne & Wear, Co. Durham and Cleveland,with the Mayo clinic figures extrapolated, Northern idiopathic dystonia in Regional 1,009 the people with gave an estimated Health Authority (NRHA) area. These calculations were based on the population by Research fi7om the county's each mid-1991 census,which were supplied statistics following in in March table. Units 1993 Intelligence the shown and are as updated and TABLE 10. Prevalence of the various forms of dystonia in the North East region based on the Mayo Clinic estimates. County (with) : Population Cleveland 550,100 Co. Durham 589,900 Tyne&Wear : 1,125,600 Northumberland : 304,700 Cumbria : 483,200 Totals =: 3,053,500

GD 19 20 38 11 16 104

BL 28 30 57 16 25 156

Index :"

" " " " "

GD

Dystonia, Generalised = BL = Blepharospasm, OMD = OromandibularDystonia, Dysphonia, SD Spasmodic = ST = SpasmodicTorticollis, WC = Writer's Cramp.

17

OMD 19 21 39 11 17 107

SD 29 31 58 16 25 159

ST 49 53 100 27 43 272

WC 38 41 78 21 33 211

Dystonia -A comprehensiveand longitudinal study in the North East of England

As previously identified on page 2, there were currently only 143 people identified in dystonia May 1993, at the time of the start of the survey, therefore it was with be little point in conducting any meaningful researchwith that there would obvious (143) 14% of the estimated population of 1009 people potentially available, only particularly as even those calculations were based on a "Substantial underestimate" (Marsden and Quinn, 1990) and there was no way of establishing any sort of known, the than those of sample more unless currently representativeness people, were correctly identified. Based on the Dystonia Society survey (TDS, 1993), already mentioned on page2, and the author's own personal experience, it was deduced that a high proportion of in in U. K. the remained undiagnosed sufferers general and within the North East in particular. Therefore it was essentialthat, before any researchcould be undertaken, somemethod identify found to more people with dystoniain the region than currently available, was be then could askedto participatein the author's proposedresearchproject. who Any epidemiologicalsurvey of dystonia would, by definition, be quantitative in nature as its purpose was initially merely to identify the actual numbers of people with dystoniawithin the NRHA (Northern RegionalHealth Authority). As previously mentioned, accessto people who have dystonia is consideredto be the primary difficulty in carrying out any sort of researchproject (Butler et al, 1998) and as the very process of identifying those people with dystonia meant first obtaining a correct diagnosis, the opportunity arose of having previously unknown (unidentified and undiagnosed)patients availablefor other dystoniaresearchprogrammes. As there were only 12 clinical neurologistspractising in the North East at the time, the averagenumber of dystonia patients registered at any neurology clinic was estimated to be relatively small. However it was known that, in practice and based on the author's personal experience,a couple of consultantshad registered the bulk of the diagnoseddystonic patients in the NRHA area,mainly due to the advent of Botulinum Toxin therapy in the treatment of dystoniaand secondarydystonic spasms. It was at this point in the negotiations with the Dystonia Society and local medical personnelthat the idea of an expandedproject basewas first mooted by Mr Maurice Hawthorne, who is an Otolaryngologist and Consultant Surgeon at the ENT Department of the North Riding Infirmary in NEddlesbrough and who runs the Botulinum Toxin clinic basedthere. The author had approachedthe headsof the two Botulinurn Toxin clinics for accessto their patients and through this initial contact in February 1993, the Cost Utility Analysis (CUA) of Botulinurn Toxin therapy in the treatmentof dystonia (Gudex et al, 1995)was started on 6th May 1993. Although the CUA was the initial research,it was always envisagedas a method of firstly expanding the author's knowledge of dystonia and secondly training him in general researchtechniquesand enabling an expansionof people with dystonia into a formal epidemiologyof dystoniawithin the North East region.

is

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART TWO: DYSTONIA CHAPTER 6 Treatments Research of the literature has shown that treatments, prior to the development of Botulinum Toxin, have proven very hit and miss regarding a stable and useful treatment for dystonia. With the exception of the use of Levodopa in the treatmentof dystonia in cases of Dopa-ResponsiveDystonia, the use of other drugs has proved have been There variable. a large number of different drugs used over the years very with differing results, none of which have proved particularly effective. Until the pathogenesisof dystonia is understood, the treatment of the majority of dystonia will be non-specific,aimedat the symptomsbut not the causeof the disease.The treatment of dystonia was often empirical and symptomatic,but there are a group of conditions which often do respond to treatment. There also are a number of conditions which often respond to specific drug treatments,which are listed below.

Conditions with a Specific Rational Therapy 1. Wilson's disease: One of the first things to be eliminated, when patients with a dystonic disorder are evaluated, is Wilson's Diseasewhere an excessof copper is stored in the body. This is eminentlytreatable,with the oldest caseon record being about 53 years of age. 2. Acute drug-induceddystonia: The acute form of tl-ýs diseaseis where a person is given treatment for sicknessand within minutes of being given an injection, the eyesare turning up into the head, often with an arching of the back, called an oculogyrie crisis. This responds very well to intravenously given anticholinergics or Diazepam and is an acute disorder, ie., respondingwell to treatment and not being long term. 3. Chronic drug-induceddystonia: Tardive Dystonia, which has only become recognisedin recent years, representsone perspective of Tardive Dyskinesia. This occurs in patients who have received long term neuroleptics, often with a psychiatric diagnosis, and may look indistinguishable from an idiopathic dystonic person, although this is a drug-induced problem. The chronic form may respondto antidoparninergics,anticholinergics,Benzodiazepinesand Baclofen, although it is the neuroleptics that have causedthe problem and it is not always an easycondition to treat.

19

Dystonia -A comprehensiveand longitudinal study in the North East of England

4. Parkinson'sDisease: Dystonia can often occur in Parkinson's Disease. It can occur in drug-naive Parkinson's Disease, where patients who have never received treatment for the condition and may have painýl dystonic inversion of the feet which might well improve on introduction of treatment. Drug-induced dystonia in the long standing Parkinson'sDiseasepatient is also a feature and is often very difficult to treat. 5. Lesions of the Basal Ganglia: Lesions of the basalganglia may often be part of the causeof herni-dystonia. A lesion or a structural cause in the opposite basal ganglia, if it is amenableto surgery, can often lead to someresolution of the symptoms. 6. Dopa-ResponsiveDystonia (DRD) : 5% to 10% of patients with childhood onset dystonia, beginning in the legs, respond , dramatically to Levodopa in small doses with no long term complications, which does often coexist with elements of Parkinsonism. AH childhood onset dystonia cases should have a trial of Levodopa as the treatment is effective indefinitely and not associatedwith the long term problems that Levodopa has in treating people with Parkinson'sDisease.There is often an inherited componentto it but it does not always expressitself and DRD is often called "Segawa'sSyndrome" in someliterature. 7. ParoxysmalKinesogenicDystonia : This form of dystonia occurs where, if the patient moves suddenly, they will adopt dystonic postures. This can occur multiple times in a day and every time they move suddenly, they will trigger the dystonia. These cases are really quite sensitive to anticonvulsants,so drug treatmentcan be very effective. 8. Paroxysmal Non-Kinesogenic Dystonia:

Its colleague is not related to movement, but if the patient has a heavy meal or is anxious or stressed or drinks alcohol, they can adopt a more sustained dystonic posture for a more sustainedperiod of time. This condition respondsreasonablywell to Benzodiazepines. 9. Sandifer'sSyndrome: Sandifer'sSyndromeis a condition peculiar to children, where after eating a meal the child will develop borborygmi and often their head will adopt the posture of a severe dystonia. The mechanismof this is thought to be relatedto oesophagealreflux of acid. By doing an operation to cure the reflux, one can cure the condition. 10. Autosomal Dominant Myoclonic-Dystonia Syndrorne: This is a mixture of myoclonus, so called jerking problems, together with dystonia which respondsquite well to alcohol. 20

Dystonia.-A comprehensiveand longitudinal studý,in the North East of England

Surgejy and OrthopaedicProcedures 1. Ablative CNS lesions Prior to 1950, people like Horsley (in 1900 he first treated hyperkinesiasby ablating the motor cortex in a boy with severe hemi-dystonia), Bucy and Case (1939) and Klemme (in 1940 he excised cerebral cortex for Parkinsonism tremor and dystonia), Putnam (in 1938 he incised the pyramidal tract in the upper cervical cord) and Walker (in 1952 he incised cerebral peduncle in the n-ýidbrain)were doing fairly crude in procedures an effort to try and stop involuntary movementscontralaterally. A lot of the early patients had Parkinsonism, or Parkinson's Disease, or post encephalitic Parkinsonisrn,and they had dystoniaassociatedwith it, but some of these patientsalso had primary dystonia and the price that all of these patients had to pay was Hemiplegia. The patients sacrificed an abnormal posture for a loss of function to a profound degree. So really it is not surprisingthat theseare not practisedanymore. However, by 1951, Meyers startedto experimentwith deeplesionsand showedthat by putting a lesion, deep down in the Basal Ganglia,the contralateral movement disorder could be improved without incurring the terrible price to pay of hemi-paresis. He showed globus pallidus lesions could reduce contralateral dyskinesias without in and spasticity weakness Parkinsonians,but with a 16% mortality. Cooper was trying to copy Meyers when he inadvertently cut the anterior carotid artery. He quickly finished the operation having sealedoff the artery. When the patient recovered.,it actually turned out that he had achieveda better result than the patients that Meyers was treating. Cooper then started performing this lesion intentionally, rather than cutting the tract, he started to ligate the arteries. Between 1955 and 1974, he did 226 chemopallidectomiesand chemothalamotomiesfor torsion dystonia. Most patients received I or 2 operations, some up to 7 in total. He retired in 1976 and reported a huge series of cases,where he had injected phenol or alcohol into the globus pallidus and into the thalamus to treat torsion dystonia. Cryothalarnectomy (freezing of the thalamus) was eventually the most favoured technique and Cooper reported that 70% of his patientsactually gained'somebenefit'. This is an unparalleled figure in the literature although several patients paid a price by having swallowing difficulties. By 1983, Andrew found that limb dystonia, especiallyhemidystonia, respondedbest and that lesioning the posterior portion of the ventrolateral thalamus (sensory relay nuclei) gave the best results. This operation was reviewed by Andrews most recently who showed that, in bilateral thalamotomies,there really is a huge morbidity and mortality; over half the patientsare left with major problems speakingand swaflowing, comparedwith only 11% for the single side operation. Andrews also pointed out that limb dystonia, and hemidystoniaparticularly, respondsbest and lesioning the particular part of the thalamus, which is a sensoryrelay station, and passeson messageto the brain, generallygives the best result.

21

Dystonia -A comprehensiveand longitudinal studv in the North East of England

2. CNS Stimulation

Cerebellarstimulation has beenattemptedby Siegfhed and Hood but by 1982, Waltz's cervical cord stimulation claimed to benefit 72% of 110 cases with generalisedand dystonias, however further studies fail to substantiatethis claim. Peripheral cervical for first torticollis was attempted in 1891 by Keen, where he divided the first surgery three cranial nervesextraspinally. Then in 1915, Taylor attempted the first intraspinal approachwhich showedbenefit in up to 80% from rhizotomy of anterior roots of C 1-3 bilaterally but with a high complication rate. Waltz in 1982 claimed amazingresults by stimulating the cervical cord. He claimed Z2 that 72% of his patients in a large seriesdid very well. After more critical evaluation, this procedure can no longer be recommended.Now peripheral surgery takes all sorts of forms. Basically it involves tackling the nervesand musclesextraspinallyat the site of the problems. Of the other problems that have been tackled, probably the main one is Spasmodic Torticollis. The idea has been to cut as many nerves as possible without leading to unacceptableside effects.A Rhizotomy sectionscervical roots as they emergefrom the spinal cord. Many patients undergoing this operation became quadriplegic, whilst others developedswallowing difficulties. By 1989, Bertrand and Molina-Negro were doing selective extraspinal denervation using intra-operative nerve stimulation to identify branchesand claim 87% successin a relatively small seriesby attacking the specific musclesthat are involved in Spasmodic Torticollis. 3. Physicalmethodsand behaviouralmodifications There are certain things that make dystonia a lot worse, ie stress,lack of sleep,having no one to talk to, isolation and the feeling of being misunderstood. There are a lot of patients where there can be a huge delay in making a correct diagnosis and this can lead to a great deal of misery and can exacerbatethe problem quite markedly. Doctors often when speaking to patients with dystonia are adopting some form of psychotherapyto make people realisethat they are not alone and although they do not know the cause,they are trying to do somethingabout the problem. 4. Relaxation therapy and self-hypnosis Relaxationtherapy can have somelimited benefitsas can self-hypnosis. 5. SensoryBiofeedbackmethods This is where people can have an EMG or muscle monitors put on their muscles feeding back to themselves,so that they can see when a muscle is becoming overactive and they can adopt strategieswhere they can relax and make the activity die down, so that the musclebecomesless over-active. As will be seen,this has not been Popularwith North East dystonia patients with only 6 having tried this techniquewith no satisfactoryresults. 22

Dystonia -A comprehensiveand longitudinal study in the North East of England

6. Mechanical devices/ braces These have had a varied responsein the past. Generally, ill fitting braces and rigid appliances, which try and constrain the movement of dystonia, can actually be harmful. However imaginatively designed devices can be very useful. positively People who use sensorytricks for SpasmodicTorticollis or Retrocollis certainly have devices where, with an appropriatebrace andjust a lightly touching device in the used occipital area, can actually receive a very slight sensory stimulus and prevent the from showing too much of a dystonic pattern. patient DruR Treatments

Systemic pharmacotherapycan overall give benefit to 30-40% of dystonic patients. Initial treatment is guided by use of drugs with low potential for adverse side effects and the anatornicdistribution of the dystonia. About 5% of patients with any form of idiopathic dystonia may experiencea spontaneousimprovement or resolution of the movementdisorder, this is most likely in the first five years and relapsesare common. 1. Anticholinergic Agents Drugs like Benzhexol or Artane may help approximately50% of children and 40% of adults and very large does (60-100 mg) may be needed, as opposed to people with Parkinson's Disease who may be given just 6 mg to help with their tremor. Improvement is independentof age of onset, age at starting the therapy, severity of dystonia at start of the therapy and aetiological diagnosis. However improvement is dependanton dosage,side effects (more prominent in adults and a function of age not serum level), peripheral (dry mouth, urinary retention, constipation and blurred vision may need co-administrationof philocarpineand pyridostigmineas muscarinic agonists) and central (may causedrowsiness.,confusion,memory impairment and hallucinations). The improvement is often independentof the facts listed above and is dependantupon the dosage,although that is very loosely related in the big trials done and some people respondto a much lower dose.The method is to start on a given low dose and to build up very slowly becausethere may be a gap of severalmonths before people respondto a particular dosage. Certainly, in the series done by Professor Marsden, a large numberof people, 65% of adults, had side effects and a large number of them were so bad they had to stop taking the drug. 2. Dopamine Agonists This includes Levodopa, which has already been discussed. There is a query about whether there is a sub-setor sub-groupof the torsion dystonias,becausethe samedrug can actually worsen symptoms in about a third of adult cases. 5% to 10% of childhood onset dystonia beginning in the legs respond to low dose of Levodopa, however it may worsen symptomsin up to 35% of adult cases. Greeneet at reported in 1987 that 12% of 41 patients who failed anticholinergic treatment respondedto dopamine agonists eg Lysuride. This is the same sort of drug as Bromocriptine, a direct acting DopamineAgonist. 23

Dystonia -A comprehensiveand longitudinal study in the North East of England

3. Doparnine antagonists

These are the exact pharmacological opposite to Doparnine Agonists, yet 20% of people will respond to these drugs. No individual has responded to both dopamine agonistsand antagonistssuggestingagainmultiple pathophysiologicalmechanismsmay be involved. Thesedrugs are either DopamineDepletors, eg Reserpine,Tetrabenazine, from the nerve before it is released where they act to actuaHyget rid of the Dopan-ýine or they are Doparnine Receptor Blockers, eg Pimozide and Haloperidol. They latch onto the receptors,post synaptically,and stop the Dopa from being active. Responseis variable generafised/ segmental = 0-78%, cranial dystonia = 3-34%, spasmodictorticollis 9-46% and writer's cramp = 0%, which just shows that it is very difficult to have a consistent scale of assessmentand assessmentcriteria vary. The side effects, certainly with Tetrabenazine, a dopamine depletor, are quite significant depressionand that is what is so limiting. In addition, with the Dopamine ReceptorBlockers, Parkinsonismmay also be a problem. 4. Other Drugs Inadequatedosing and inadequateduration of treatment may make a drug appearless effective than it reafly is. Here is the sort of treatment where people may rather idiosyncratically respond. These are not the first line drugs becausethey are often, in many cases,not very effective. Baclofen : The physiotherapistsdeal with this drug as a spasmolytic. It may be effective in about 20% of generaliseddystonia and is often successfulin occasional segmentaldystonia. Benzodiazepines: There is no evidenceone is better than the other. Clonazepmnis the drug that people tend to have used a lot with 15% of patients responding. Carbamazepine,an anticonvulsant,helpsbetween10% I% of patients. -I Alcohol : With the use of alcohol, intravenous infusion may help to reduce severe drug-unresponsive Spasmodic Torticollis, although this is clearly not a practical treatmentto be walking around with an alcohol infusion running. Lithium: This is a very toxic drug, has a limited use and may help blepharospasmwith oromandibulardystonia, ie., Meige's Syndrome. 5. Triple Therapy This is used in children and especiallyadults with severe(sometimeslife-threatening) dystonia not responding to monotherapytrials, where the muscle spasmis so severe there is breakdown of muscle, increased body temperature, exhaustion, respiratory difficulties. It comprisesof Tetrabenazine(75 mg), Pimozide (up to 12 mg daily) and Benzhexol (up to maximum tolerated and titrated against Pimozide). In a series reported by Marsden et al in 1984 this helped I of 2 children and 9 of 12 adults with severedystonia

24

Dystonia -A comprehensiveand longitudinal study in the North East of England

in conclusion, a small number of patientswith dystonia may be helped with a specific treatment for a specific cause of the condition. Many patients with generalised dystonia may be helped by systemic pharmacological therapy. The treatment is empirical and roughly follows a 'ladder' schemewhere the 'least toxic-most likely to be successfulas monotherapy' is at the bottom and the 'most toxic-polytherapy' is at the top. In drug treatment the secretis to take a single drug to its tolerated limit (in most cases)and not to increasedosestoo quickly. All childhood and adolescentonset cases of dystonia should be given a trial of Levodopa treatment first. Centrally placed CNS lesions may be very effective in selectedcasesbut surgery is regarded as the 'last gasp' treatmentin most centres.Peripheralsurgicalprocedureshave now been supplantedby Botulinum Toxin therapy.

The Introduction of Botulinum Toxin Therapy

The advent of Botulinum Toxin therapy has revolutionised the treatment of dystonia. In May 1993, there were only 149 people who were getting Botulin um Toxin therapy in the North East of England, whereasby May 1999,6 years later,' the number was 722, a3 85% increaseor an averageincreaseof 64.1% per year. Botulinurn Toxin injections can relieve the symptoms of dystonia and this is now considered as the best treatment for many adult onset focal dystonias such as blepharospasm(Grandas et al, 1988), writers cramp (Cohen et al, 1989) spasmodic torticollis (Stell et a], 1988) and spasmodicdysphonia(Ludlow et al, 1989) and it was tlýs aspect in the treatment of dystonia which was the primary area covered originally by the research project when it commencedin 1993 (Gudex et a], 1995,1997 and 1998). C' research into dystonia is more active now than ever before. As a result, ... physician awarenesshas increased greatly. A once obscure condition which was usually thought to be psychological in origin is now recognised as a relatively common reason for referral to movement disorder clinics. Although treatment remains difficult and oftenfnistrating .... thesefactors allow individuals who carefor these unfortunate patients to provide them with some hope that relief ftom their considerabledisability eventually may be realised. " (Riley and Lang, 1988) There is a clinical syndromecalled 'botulism, where a bacteria 'Clostridium Botulinum' overgrows in the gut. There was an outbreak in Preston,in the North West of England a few years ago, where cans of Hazel Nut puree which, at their site of origin, had becomeinfected with this anaerobicbacteria. Being in an enclosedcan, they cameto multiply and survive very nicely. Then they were put in pots of yoghurt which were then sealed. Unfortunately this causeda large outbreak of clinical botulism. The cases presented with difficulty with swallowing, difficulty with speaking and respiratory problems. They required intensive care and management, but with appropriate treatment, including antibodies and ITU type approaches,they recovered quite well. These toxins, which are produced by these micro-organisms,are potent neurotoxins and very small quantities of this agent are needed to cause the above type of symptoms.

25

Dystonia -A comprehensiveand longitudinal study in the North East of England

There are 7 scrotype separatechemicals,which this group of bacteria produce. In terms of clinical use, it is serotypeA which is currently used, although other serotypes Z) investigated for being their clinical usefulness. It is such a potent toxin that are absolutely minuscule quantities of this agent are used. It effectively weakens the muscle into which it is injected and it can relieve spasmsand involuntary movements. The quantities that are used do not usually causeprof6und weakness. There is only a is it the that thus of motor end affected muscle plates are and able to percentage dampen down the over activity, rather than causing actual weakness. One of the fiindamental problems is it is an organic compound and that a proportion of people who are treated can develop antibodies. The effect of the antibodies is that they neutralisethe effect of the toxin. It effectively controls dystonia for between 8 to 12 weeks. It is a localized treatment with minimal systemiceffects. It can be used on an out-patient or in-office procedure and it controls or relieves symptoms in up to 98% of patients. Everyone who is involved in treating dystonias with Botulinum Toxin is simplifying their injection techniquesand as the numbershave increasedso has the expertisein adrninisteringthe injections. The following chart gives where the injections are usually placed. TABLE 11 : Injections Sites for Spasmodic Torticollis

Headneck position

Muscles involved

Torticollis

SCM contralateralto the direction of turning, Ipsilateral trapeziusand/or levator scapulae

Laterocollis

Ipsilateral spleniuscapitis, trapezius,scalenusand / or levator scapulae

Retrocollis

Bilateral spleniuscapitis, semispinalisand longissimuscapitis

Anterocollis

Bilateral SCM, scalenilsubmentalcomplex

In ordinary torticollis, the Stemo-Cleido-Mastoid(SCM) is injected, contralaterallyto the direction of the turning and either the ipsilateral trapezius or the bilateral trapezii, dependingon the relationshipsof the movementsaround the neck. The spleniuscapitis is often injected as well becausethat muscle is often active in SpasmodicTorticollis. For Retrocollis, bilateral spleniuscapitis musclesare injected and it is slightly deeper is difficult isolate difficult Anterocollis to treat, these to very and more muscles. head injections. do bilateral SCM The problems on occasions. scalenil cause requiring I-

26

Dystonia -A comprehensiveand longitudinal studý,in the North East of England

PART THREE:

METHODOLOGY

CHAPTER 7 Awareness Raising As can be seen from the evidencein the literature review, no one knew how many casesof dystonia existedin the U.K. and, at the time of the start of the researchin May 1993, there were only 143 people with dystonia,known in the region. Therefore the initial problem was obviously going to be to see if sufficient numbers of people with dystonia could be correctly identified and this was predicted to be a mammoth task. Therefore a seriesof awarenessraising campaignswere launched from 1993 to 1998, ie., during and throughout the entire researchprocess. The author was involved in giving all or part of a numberof separatelecturesduring this period as follows :Twelve AwarenessRaising Events in the LJK 1.14th July 1993 : Dystonia AwarenessRaising Day held in the Lecture Theatre at Hunters Moor Hospital, Newcastle,UK. 2.20th April 1994 : Dystonia AwarenessRaising Day held in the Lecture Theatre at Hunters Moor Hospital, Newcastle,UK. 3.31st March 1995 : An International Workshop on the use of Botulinum Toxin for the treatment of dystonia.held at ManchesterUniversity, UK. 4.9th April 1995: The Dystonia Society Conference at Warwick University, UK. 5.24th - 28th June 1996 : The First World Congress in Neurological Rehabilitation held in Newcastle, LJK.

6. September 1996: The AwarenessRaising Campaignin Darlington, LJK. A total of 101,766 people living in 45,383 houses within the postal districts of Darlington, Co. Durham, ie DLI, DL2 and DL3, were given a pre-addressed leaflet describing dystonia and asking them to send it back to the author, if they felt they exhibited any of the symptoms described on the leaflet. A total of 41 people replied who were then visited and exarnined by a qualified neurologist.

7.30th January 1997 :A Newcastle.

Dystonia Orientation Day at Hunters Moor Hospital,

8.31st January 1997 :A Dystonia Orientation Day at the North Riding Infirmary, Middlesbrough.

27

Dystonia.-A comprehensiveand longitudinal study in the North East of England

9.23rd to 27th March 1997 : The "Health and the Musician" Conference held at York University. 10.28th May 1998 : "The extendedrole of the nurse in a Botulinum Toxin clinic " at the North Riding Infirmary, NEddlesbrough. 11.22nd September 1998: The North Devon District Hospital, Barnstaple,Devon. 12.3rd December 1998 :A Dystonia Study Day held in the Conference Room at Hunters Moor Hospital, Newcastle.

Four AwarenessRaisin Events abroad 13.15th June 1995: The Botulinurn Toxin Conferencein Munich, West Germany. Two poster presentationswere made at the above international conference. One was entitled " The social and economic implications of dystonia " and has since been publishedin the EuropeanJournal of Neurology; 3: 79. The secondwas entitled " An epidemiological survey of dystonia in the North East of England " and was since publishedin the EuropeanJournal of Neurology; 3: 28. 14.9th - 11th October 1996 : The Third International Dystonia Symposiumheld in Nfiami, Florida, USA. Two lectures were given, which have since been published as chapters and printed in Advancesin Neurology, Volume 78: Dystonia 3. They are " The Epidemiology of the Primary Dystonias in the North of England " given by Dr Phil Duffey and " The Socio-EconornicImplications of Dystonia " given by the author. 15.8th and 9th November 1997 : The National SpasmodicTorticollis Association Symposiumheld in Nashville, Tennessee,USA. A joint lecture with John Whitaker, the Outreach Dystonia Nurse Practitioner, was given. The author's lecture was entitled " Some Social and Economic Implications of SpasmodicTorticollis in the North East of England." 16.7th and 8th November 1998 : The National Spasmodic Torticollis Association Symposium held in San Diego, California, USA.

A repeat of the previous year's series of lectures with John Whitaker, the Outreach Dystonia Nurse Practitioner. The author spoke again on "Some Social and Economic Implications of SpasmodicTorticollis in the North East of England."

28

I

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART THREE:

METHODOLOGY

CHAPTER8 Data Collection and Instruments Data Collection

Only three sourcesof dystonic patientswere initially availableto the author in 1993 :a) the register of Dystonia Society membersin the northern region, then defined as the five northern countiesof Cumbria,Northumberland,Tyne & Wear, CoDurharn and Cleveland,which had the sameboundariesas the NRHA (seenote below). b) the patients registeredat the Movement Disorder Clinic of Hunters Moor Regional Rehabilitation Centre OHMRRC)in Newcastle. c) the patients registeredat the Botulinum Toxin clinic of the ENT Departmentof the North Riding Infirmary (NRI) in Nfiddlesbrough. Note : By April 1994, the NRHA's boundaries had been changedand the southern part of Cumbria was removed and Yorkshire was added and the Northern and Yorkshire Regional Health Authority (NYRHA) was formed. Henceforth all epidemiological researchinto Prima!y Dystonia was restricted to the four counties of Northumberland, Tyne & Wear, Co. Durham and Cleveland. Researchinto any social or economicimplicationswas not however restricted geographicallyin the sameway. Having establishedthe number of people with dystonia registered within these three primary sources,the rest of the researchwas initially carried out by a combination of interview, questionnaireand researchthrough the clinical notes of the subjectsat the two medical venues. The only criteria used in initially selecting subjectsfor the study was their referral to, or participation in, these two clinics administering Botulinurn Toxin (Bot. Tox.) injections in the area. Subsequentlya third Bot. Tox. clinic at the SunderlandEye Infirmary (SEI) was usedfrom 1995 onwards. However applications were made (and approved) to the Local Research Ethics Conunittees(LREC) in January1994, once the CUA fieldwork had beencompleted,to expand the ESD research in an attempt to identify further previously undiagnosed subjects. This was accomplishedby dividing the data collection into distinct phases. The first phaseincluded personallyattending the relevant clinics at FIMRRC, NRI and SEI and interviewing all the patients attending. A very small number of patients were screened out of the study from the clinics at NRI and SEI, if their injection of Botulinurn Toxin was post ophthalmic operation and non spasmodic. In particular, those patients who did not have any dystonic spasms and where either M. S. or Spasticitypatientswere removedfrom the lists.

29

Dystonia -A comprehensiveand longitudinal study in the North East of England

All clinical data was collected and processedon a Clinical Information Form (CIF) and no verification of diagnosis was made until the differentiation between primary and dystonia due to other neurological conditions was confirmed by and spasms secondary the Neurological Registrar. The records of other hospitals in the area were reviewed and the examinationof casenotes was not restricted to those coded for dystonia. The review also included case notes coded for any unspecifieddisorder or movementgait not attributed to a specific disease. An example of how detailed this work becameand how this complicated procedure was accomplishedcan be seen by a quote from the Darlington Memorial Hospital (DMH) study (Butler and Duffey, 1996b),below: cca total of 20 visits were madeftom 8th Fehruary to 8th August 1995 during which time all 665patients who had attendedthe DMH neurology clinicftom 41hSeptemher 1991 (when computer records were commenced)to 29th March 1995 (when ICD 10 codeswere introduced) were screened,a total of 4.5 years. A total of 10 primary and 10 secondarydystonia caseswere identified together with ajurther 4 casesof hemifacial spasmftom the researcher'scoding (ICD 9) of all 665 cases." During 1995, the Sunderland Eye Infirmary (SEI), Sunderland General (SGH), NEddlesbroughGeneral(MGH) and the Dryburn Hospital (DHD) in Durham were also degrees and visited varying of successaccomplishedfurther identification of dystonic patients. For example,all casesof dystonia attending SEI have since been identified and staff continue to monitor all new patientsattendingtheir Botulinurn Toxin clinic. 1996 saw the expansion of this programme to include continuous monitoring of all neurology clinics in the North East to establishall new patients attending, principally the Royal Victoria Infirmary (RVI) and the GeneralHospital (NGH) in Newcastle. Liaison with the Family Health Services Authority (FHSA) in South West Durham establisheda databaseof the 3 10 individual doctors practising in County Durham. A series of GP Focus Groups establishedthe best methodology of contacting and recruiting GP's onto the ESD. Dr Phil Duffey, at that time Hon. Senior Registrar at the RVI, undertook to contact those within the Darlington catchment area, ie with a Post Code DL I, DL2 or DL3, and visited them during 1996. After discussionswith several epidemiologistsand other experts in the field, it was decidedthat Darlington, which is a town of 101,766people, should be the subject of an intensivecampaignin 1996 to establishthe prevalenceof dystonia witl-ýn its known population. This was achieved by a combination of all the above phases,with the added impetus of two other distinct campaigns, run in conjunction with TDS throughout the North East. The first involved a publicity campaign whereby all the local media, including radio, television and the presswere contacted and persuadedto run a short but intensive publicity campaign about the Epidemiology in general and diagnosisof those outside the net from the previous stagesin particular, ie those as yet undiagnosedor not registeredwith a neurologist. This type of advertising has proved very successfuland has resulted in some spectacularsuccesseswhich will be discussed later in the thesis.

30

Dystonia -A comprehensiveand longitudinal study in the North East of England

The secondcampaignwas a mail shot of all 45,383 homes in the Darlington area, in which a short but effective leaflet askedhouseholdersto identify a number of specific muscular spasms,from which the screeningprocessthen commencedto diagnoseall those potential people with a dystonic spasm.This took place during the autumnal months of 1996 and enabledthe researchteam to attempt 100% validation within a relatively small geographicalarea. Finally, the researchteam attempted to identify any other dystonic subjectsthrough tracing a numberof known medicationsback from the subscriber/ dispenserto the purchaser. This inevitably threw up a number of other neurological disorders which required screening out, but this, the final and most difficult stage, was effectively completed by the end of 1998. The ESD will now continue, using a numberof different sources,at least until 2003 and hopefully beyond. Instruments The appendicesinclude copies of all the actual questionnairesused in the research includes and study a number of documentsusedinitially to gain informed consentfrom the patients, however it is important to realise that a number of different methodological techniqueshave been used throughout this research study and these have often beencombinedto produce the overall results. All questionnaireswere initially numberedfrom I through to 9 inclusive, however this 0 to 99 to enableother instruments within the samearea was changedfrom 01 through of study to be inserted, as required. The questionnairesused are listed below for referenceonly and detailedunder their own individual sections.

Questionnaire No's 01 to 13= Qualityof Life andHealthStateQuestionnaires Chapter9 outlines thesequestionsin full. QuestionnaireNo. 20: Demog Dystonic Profile Questionnaire. and graphic The questions were designed by the researcherand were agreed by the Dystonia Society's research sub-committee in March 1993 (Focus Group 1993). A postal version of the questionnairehas beenusedwhere the patient lived outside the NYRHA area,ie the West Indies, the WesternIsles andLondon to namebut three participants. The appendicesshow the actual demographicquestionsand answerssheetsusedin the interview, which usually took place in the patient'shome. It was soon realisedthat the clinic was not the best place for the interview to take place as the time availablewas too short and the circumstancesof the clinic often too traumatic for reliable and indepth answers. The questionnaire was designed to obtain purely demographic information as well as provide a demographicand dystonic;profile of eachsubject. All the information was of a purely factual nature and therefore required no validation, except that in a number of areas, the patient's memory of events was verified by referenceto, and inspectionof, their clinical records,casenotes or hospital files.

31

Dystonia -A comprehensiveand longitudinal study in the North East of England

In total, 473 interviews took place over a4 year period. The questions were divided into relevant sectionsas follows :a) Demographic clata,including social and economicinfon-nation. Geographicallocation - Post Code for determiningcounty, district and postal sector. Age - date of birth, confirming that the Clinical Information Form (CIF) was correct. Gender- male or female. There were no specificquestionsregarding ethnicity. Marital status- noted single,married,divorced, separated,cohabiting or widowed. Number and genderof children, including eldest's and youngest's year of birth. Social Income Group - researcher'splacementbasedon occupation and status. Current Income - patient only (family incomewas noted where no income existed). Number, range and amount of any benefits,allowances,pensions,etc. b) Dystonic Profile Current (and any other) Hospital Registrations- comparedwith CIF. Travel time and method to and fi7omcurrent clinic - of patient and partner / friend. Date (as near as possible)of Onset- subjectivewhich was then comparedwith CrF. Date (as near as possible)of Diagnosis- which was then comparedwith CIF date. Clinical Diagnosis(by interview and observation)- confirmed with CrF diagnosis. Dystonic type - confirmed with CrF diagnosisfrom clinical records. Aetiology - mainly from CEF,obtainedfrom clinical notes. c) Other Medical Information Possibletrigger - subjectiveopinion basedon past events Family history - in particular familial dystoniaor other neurological disorders. Current medication,dosageand frequency- potential side effects. Comorbidity - interview with subject,confirmedwith CIF. Previoustreatments- from subject'smemory, confirmedwith CIF. Side effects from previous treatments- including duration. Botutinum Toxin injections - number,frequencyand side effects. QuestionnaireNo. 21 : TDS Questionnaire. These were a number of questionsspecifically regarding the Dystonia Society, which had designed if be to the or a member not were gradually ornitted subject was not heardof TDS before. They were designedto obtain factual information on :First contact with TDS - where and how Contact with other people with dystonia- frequency,membersor not Possibility of starting a counsellingservice (which was eventuallystarted in 1995) Publicity - where first heardof TDS to measureeffectivenessof awarenessraising Membership- current, dropped out, etc., plus other voluntary organisations Which SHG - NE, Cumbria,Yorkshire, others in UK, or on the mailing list Newsletter - most read part and why National and local aims - open endedquestionsabout the future.

32

Dystonia -A comprehensiveand longitudinal study in the North East of England

Questionnaire No. 25 : Demog Changes Questionnaire. graphic

This questionnairewas administeredat the end of the study (sent out with No's 80 & 81) to each subject to determineany changes,which were then included as part of the SPSS Coding for QuestionnaireNo 20, with the changesnoted. It was designedto form part of each subjects' demographicprofile and related specifically to those areas of factual information which could possibly have changed during the period of the study, ie ;a. Employment b. Income and / or Benefits c. Time off work, if appropriate d. Changeof hospital and method of travel or distanceto and from the new clinic. NB : Any changesin dystonic spasmswere noted as part of the ESD on the CIF during the study. Of the 199 active participantsin the CUA, only 9 (4.5%) had changedclinic during the period 1993 to 1994. With one exception, which is related to a geographical relocation, these were due to a referral from the general Movement Disorder clinic at HMRRC to the specific treatment available for Spasmodic Dysphonia and Oromandibular Dystonia at the ENT clinic at the NRI in Middlesbrough. However by the end of the study the number of this type of hospital changehad risen to 23, but 258 No 25 Questionnaireswere completed in total, noting other changesduring the study period.

QuestionnaireNo, QuestionnaireNo. Questionnaire,No. QuestionnaireNo. QuestionnaireNo.

30 40 50: 60 70

Clinical Profile of Dystonia Ouestionnaire. Torticollis Questionnaire.(attachedto QuestionnaireNo. 30) Livinsg with Dystonia Questionnaire: A PUchological Profile. Impact of Dystonia Questionnaire. Prima!y Carer'sQuestionnaire.

Chapter II details all of thesequestionnairesin full. QuestionnaireNo. 80 : EnvironmentalQuestionnaire. This questionnaire,as designedby the researchteam, was a result of all the questions which remained unanswered after the first three years of the research project. A number of specific questions,which related to areasprevious unasked,had cropped up during demographic profile interviews with the patients and had been noted by the author or the Neurological Registrar. The whole environmentalissuehad been raisedby a TDS memberwho had developed an interest in the effect that environmental substanceshad on her dystonia, also a number of patientshad indicated, during their interviews, that certain foods madetheir spasmsworse. It was administeredat the very end of the researchstudy and was sent to every participant who had been registered in the ESD. A total of 300 completed questionnaireswere returned.

33

Dystonia -A comprehensiveand longitudinal study in the North East of England

The questionnairewas divided into 7 sections--

1. GeneralQuestions- eg.,favouredhand/ foot, thyroiddisease,life patterns. 2. Effect on spasms- of smoking,gas,sound,light, water,differentactions,etc. 3. ChemicalErposure-6 dichotomousvariables 4. Allergic reactions- 17dichotomousvariables 5. Food mid drink - listedaremosttypesof food anddrink inhalants 6. OtherEnvironmentalsubstances natural andchemicals days bad ? 7. Final Comments some why are good are and others QuestionnaireNo. 81 : Diagnostic Questionnaire. This questionnairewas designedby Dr Phil Duffey, who was at that time Hon. Senior. Registrar in Neurology at the RVI, as a vehicle to determine which and how many medical specialistshad been seenby the subject before a correct diagnosis had been least Remember " 5 66.7% the that evidence needed at made. of all sufferers consultations before diagnosis and 65.7% were misdiagnosedat some stage. " (TDS, 1993) In Dr Duffey's own words on the questionnaireitself :"I hope that the itiformation you have provided may make the diag7zosisoffuture patients with dystoilia easier. Dystonia can be very difficult to diagnosew7d it is not my hitetition to imply criticism of those involved in your particular case. Hindsight isawonderful tool! " It had been discoveredthat the information was not always available,nor accurate,in the patient"sclinical notes. This instrumentwas also used to determine how many and which alternative treatments had been sought before, during or after normal medical therapy. This questionnairewas issuedwith QuestionnaireNo. 80 at the end of the study, but a total of 361 were returned, 61 more than QuestionnaireNo. 80. Finally the date of this questionnaire was also used to determine the length of participation in the study and the duration of eachsubject's personalinvolvement. Questionnaire No's 91-94 : Dystonia Nurse Practitioner P[qject.

This series of questionnaireswere designedby the DNP researchteam, consisting of the author, John WMaker, the DNP, and ProfessorMike Barnes. A total of 86 x No 91,77 x No 92,76 x No 93 and 76 x No 94 were eventuallycompleted. Although this thesis will not duplicate the work carried out under the DNP Project, it is necessaryto understand what was asked and why. Therefore Chapter 13 will duplicate a number of points alreadypublished,however the questionnairesthemselves were designedas part of the Monitor / Evaluator's role in monitoring and evaluating the work carried by the Dystonia Nurse Practitioner during his two year contract. Thesewill be describedin detail under Chapter 13.

34

Dystonia -A comprehensiveand longitudinal study in the North East of England

Clinical Infon-nationForm (CIF). The Clinical Analysis Form (CAF) was designedand used by the researcherand the Neurological Registrar to record certain details from each patients' clinical records or hospital notes for the CUA research. It had spacefor up to II sets of questionnaires matchedto the injection date, including Bot. Tox. dosageand the number of injection sites. All data was then transferred onto SPSS.After cleaning the data by frequency analysis,any anomalieswere recheckedwith data on the CIF or CAF. If required, the files were also re-examinedto check the accuracyof any particular entry. The Clinical Information Form (CIF) has been modified fforn the CAF, removing the for detailed Bot. Tox. injection information in favour of the information to be need in used the ESD generally. It has spacefor basic demographicinformation, including confirmation of DoB, as well as the patient's exact clinical diagnosis with dates of onset and diagnosis. Details of comorbidity and current medications were also obtained from the records, if available. Both forms are shown in the appendicesand the OF is still being used in the ESD researchproject as administeredby the author and funded by a grant from the National Lottery CharitiesBoard (NLCB).

Validation Validation of all the survey data took severalforms :1. Each case file (once identified) was examinedby a suitably qualified neurologist (Dr Phil Duffey) to ensurethat a correct diagnosishad been made by the author. Where necessarya personalvisit was madeby Dr Duffey to the subject's home. 2. A differentiation between primary and secondarydystonia and other neurological movementdisorderswas madeby the author and confirmed by Dr Duffey. 3. The diagnostic data was then presentedto an independentsource for verification and feedback,usually Mr M. R.Hawthome or ProfessorM. P.Barnes. 4. Repeated application of the instruments (questionnaires) determined their reliability, enabled an assessmentof change over a defined period of time and allowed comparisonwith both internal and externalcontrol subjects. 5. External controls (not receiving Bot. Tox. therapy) were matched in age, gender and type of dystonic spasm from the Dystonia Society membership. A small number of controls were selectedto participatewho were totally non-dystonic,but who matched other similar dystonic symptoms,ie., GeneralisedMS, UInar Nerve Palsy,Hereditary SpasticParaplegiaand Carpel Tunnel Syndrome. In the CUA, externalcontrols completedn.l.t. 5 questionnaires,relating to two full treatment cycles, as did full participants in the study. The other non-dystonic controls completed all the remaining questionnairesfor eventual comparisonwith similar dystonic symptoms such as GeneralisedDystonia, Arm Dystonia, Leg Dystonia and Writer's Cramp respectively(see5 above). 35

Dystonia -A comprehensiveand longitudinal study in the North East of England

6. The technique known as capture-recapture(Laporte, 1994) was used to cross referenceeach new subjectand check if he or she had already been identified from other sources.

The total number of sourceswas then noted, as below :CHART

12. Sources used in correctly identifying

ESD Sources Medical 1

Establishment

Bot. Tox clinics

I Neurology

Depts

I

G. P. referrals

of Information

The Dystonia 1

I Membership

I

I

ENT & Eye Clinics

Society files

TDS Newsletter

Awareness I

Training

and diagnosing each subject.

Raising Seminars

36

I

Private

Sources

I

1. Word

of mouth

I

I'D arl in gton' Mai Is hot

I

The Media Confidential

sources

I I

I

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART THREE:

METHODOLOGY

CHAPTER 9 The Cost Utility Analysis This chapter relates specifically to the combined 'Quality of Life' and 'Health State' Questionnaire,which was numberedfrom 01 to II in the Cost Utility Analysis (CUA) Toxin Botulinurn therapy and was then numbered from 12 to 13 in the of Epidemiological Survey of Dystonia (ESD). There was also a final section at the end which related specificallyto Dystonia Self-Perceptionquestions. The CUA took place from May 1993 until June 1994, whereas the ESD was continuousuntil the final date of May 1999 and related to a total of 6 years continuous in 409 total these only although a of subjects study, completed questionnaires the ESD at the beginning and 319 subjects at the end of the study. 199 subjects completed 1,243 questionnairesin the CUA, thus a grand total of 1,772 of these questionnaires were completed and returned during the duration of the study. During the CUA, this instrument was used as a postal questionnaire issued at the beginning (beg : Q.01), middle (mid : Q.02) and end (end : Q.03) of each patient's injection cycle, roughly every six weeks. During this particular project, the minimum number of cycles required was 3 (ie 7 questionnairesrepeated), but a number of patientscompletedup to 5 cycles(ie II questionnairesrepeated)as shown below :TABLE

13. Sequence of CUA questionnaires used during injection cycles.

Cycle Q.01 Q.02 Q.03 Q.04 Q.05 Q.06 Q.07 Q.08 Q.09 Q.10 Q.11 Ist beg mid end 2nd beg mid end 3rd beg mid end 4th beg mid end 5th beg mid end] During the ESD, the sameinstrumentwas issuedto all consentingsubjectson entering (QuestionnaireNo 12) and at the end of the study (QuestionnaireNo 13) and the time betweencompleting thesequestionnairesvaried from 0.5 to 3.5 years. The EuroQol Questionnaire: Questions01 to 06. This instrument has been used in severalmember states of the European Community and results can be comparedwith other researchprojects within the EEC. It was used in this study as a direct result of the collaboration with the Centre for Health Economics at York University. During the CUA it has proved to be very compatible with the SF36 (Butler et al, 1995), as has also been demonstratedby other research projects (Hollingworth et al, 1995).

37

Dystonia -A comprehensiveand longitudinal study in the North East of England

The use of generic health statusmeasuresis desirablebecausethey enablecomparisons of results from treatment of one patient group with those from treatment of other patient groups or from the general population (Guyatt et al, 1991). The EuroQol (EuroQol Group, 1990) is a generic measurethat can generate an overall index of health-relatedQuality of Life (HR-QOL), which can be used in a Cost Utility Analysis (Hurst et al, 1994 and Brooks, 1996). When completing the EuroQoI instrument, respondentsdescribetheir own health on 5 dimensions,eachwith 3 levels of severity. The 5 dimensionsare :1. 2. 3. 4. 5.

Mobility Self Care Usual Activities Pain or Discomfort Anxiety or Depression

The 3 levels of severity are none (1), moderate(2) and severe(3). The 5-dimensional and 3-level structure of the EuroQol classification describes 243 composite health states. The social tariffs used here were elicited from a representativesampleof the LIK population (n = 2997). Two sets of such health states preferences,the so-called 4socialtariffs', were available. One tariff - the 'interim UK tariff' (NIVH, 1994) - is basedon the visual analoguescale(VAS) method of valuing health statesand the other data 'Al (Dolan based full 10 1995) the tariff' the et and a al, on population study is duration (TTO) This (Dolan 1996). the time-trade-off method et al, year method requires respondentsto choosebetweentwo certain health profiles : either to live in a less-than-fullhealth state for a defined period of time (in this case 10 years) and then die, or to live in full health for a period of 10 years and then die. The period of time in full health is changeduntil the respondentis indifferent between the two options. In both tariffs, health state preferencesrange from 100 (full health) to 0 (death), with negativescoresfor statesconsideredworse than death (Gudex et al, 1997). The SF 36 Health Status Questionnaire: Questions07 to 16. The anglicisedversion has been previously used in various surveys in the UK and the results can be comparedto a norm (Jenkinsonet a], 1993). The project's use of SF36 was registered with the International ResourceCentre for Health Care Assessmentin Boston, Massachusetts,USA on 26th April 1993. The instrumentwas conceptualisedas a" generic measureof health conceptsrelevant It provides a comprehensive, across age, disease and treatment groups. from health the patient's point to psychometrically sound and effective way measure of view by scoring standardised responses to stwidardised questions (Ware and Sherbourne,1992). According to the authors of the instrument: " standardisation of There SF 26 is interpretation the possible. scales content and scoring what makes of are at least two good reasons to adhere to the standards of content and scoring described in this manual. Eirst, they are most likely to produce scores with the same Outcomes Study Medical here in those reliability and validity as and other reported (MOS) publications. Second,comparisonsof results across studies are madepossible to the benefit of all who use thesecontent and scoring standards. " (Ware et al, 1993) 338

Dystonia -A comprehensiveand longitudinal study in the North East of England

The SF36 (ie short form with 36 questions) is designed to measure eight health conceptsand a self reported healthtransition. The standardisedscoring system(Ware, 1988) yields a profile of eight scalescoresas follows :PF : PhysicalFunctionin ie the extent to which health limits physical activities such , as self-care,walking, climbing stairs, bending, lifting and moderate and vigorous exercises. RP : Role Functioning - Physical,ie the extent to which physical health interfereswith daily including less than wanted, or other activities, work accomplishing limitations in the kind of activities or difficulty in performing activities. BP : Bodily Pain, ic intensity of pain and effect of pain on normal work, both inside and outside the home. GH: GeneralHealth, ie personalevaluation of health, including current health, health outlook and resistanceto illness. VT : Vitali! y, ie feeling energeticand full of pep versusfeeling tired and wom out. SF : Social Functioning, ie extent to which physical health or emotional problems interfere with normal social activities. RE : Role Functioning - Emotional, ie extent to which emotional problems interfere daily including decreased time spent on activities, work or other activities, with accomplishing less and not working as carefully as usual.

MH : Mental Health, generalmental health, including depression,anxiety, behaviouralemotional control, generalpositive effect. FIT : Reported Health Transition, evaluationof current health comparedto I year ago. The Dystonia Self-PerceptionQuestionnaire: Questions17 to 24. These questionswere designedby the researcher,as a direct result of the March 1993 Focus Group, to obtain the subject'sown qualitative view on their dystonic spasms,its presentationand their relationshipto other people. The researchsub-committeeof the Dystonia Society had a direct input, but the Focus Group, madeup of people who had dystonia, was the guiding force behind this concept and each section contained open ended questions to allow all respondentscomplete freedom in their answers. It was also designedso that the results could be both qualitative and quantitative. It was also scored and coded for SPSS by Dr Jay Holland, Psychiatric Registrar at the RVI in Newcastle, basedon a7 point scale, which was designedto evaluate any positive or negative changesover time as a consequenceof the intervention of Botulinum Toxin therapy. Extremely positive Slightly negative

3= Slightly positive

2= Positive 4= Neutral 6= Negative

7= Extremely negative

39

Dystonia -A comprehensiveand longitudinal study-in the North East of England

PART THREE:

METHODOLOGY

CHAPTERIO The. Epidemiology of Primary Dystonia An Epidemiology of Primary Dystonia (EPD) has already been written and published by Dr Philip Duffey, the author, Mr Maurice Hawthorne and Professor Michael Bames in Advances in Neurology, Volume 78, Dystonia 3, Chapter 13. However the enclosed, although partly duplicating this work, also describes the detailed methodologyin obtaining the original information. The main difference in obtaining this information, as opposed to all the other information obtained, was in determining who had a 'primary' dystonia, which was described originally as being different to other forms of dystonia only in its cause. This was not always clear and therefore where there was any doubt as to cause,then it described was not as primary. In other words, only those caseswhich were definitely primary were included in this written work. The methodology by which all dystoniasare identified has changedslightly since that time, due to the introduction of the "new". classification of dystonia, which was first introduced at the 3rd International Dystonia Symposiumheld in Nfiami, Florida, USA in October 1998. As previously described,prior to this conference,all dystoniaswere categorised in- two basic ways, either primary (idiopathic, familial and sporadic) or secondary (symptomatic with a known or assumed cause). Since then the categorisation has been expanded into a) primary dystonia, b) dystonia-plus, c) secondarydystonia and d) heredodegenerativediseases. These have been previously describedpreviously and therefore are'not reproducedhere. The catchment area for this study comprised the counties of Northumberland, Tyne and Wear, Durham and Clevelandin the north-eastof England. The region contained both rural and urban areas with major conurbations existing around the cities of Newcastle, Middlesbrough and Sunderland. The samplepopulation was calculatedto be 2,605,100 (United Kingdom Office for National Statistics, Estimated Residential Population NEd-1995). The surveywas divided into three phasesthat have run fi-om 1993 onward. In the first phase the records of neurologists, and otolaryngeal and ophthalmic surgeonsin the region were reviewed. The examination of case notes was not restricted to those coded for dystonia; it also included case notes coded for any unspecified disease. Added to this list of patients were those known to the Dystonia Society, a charitable organisation representing individuals with dystonia and campaigning for increased awarenessof the condition.

40

Dystonia -A comprehensiveand longitudinal study in the North East of England

In the second phase of the study measureswere taken to heighten awarenessof dystonia in the region; articles describingdystonia were placed in local newspapers,a series of radio discussionson the subject were given and on two occasions a short video sequenceillustrating the more common forms of dystonia was screenedon the regions' independenttelevision channel. In addition, seminarsfor family physicians and other medical practitioners on the subjectsof dystonia and the use of botulinum toxin were organised,in conjunction with the Dystonia Society in 1993,1994,1996 and 1998. Finally, a postal surveywas undertakenin a well-defined area within the region already exposed to phases one and two of the study. A brochure containing pictorial representationsof the various focal dystoniasand information regarding the conceptof dystonia was delivered to 45,383 householdscontaining 101,766 individuals. The targeted addresseswere containedwithin three postal codes; most were within a single The demographic town boundary, its suburbs and neighbouring villages. characteristicsof this community were consideredto be representativeof the region as a whole. The aim of the postal survey was to gauge, albeit crudely, whether a significant number of individuals with dystonia remainedto be identified after the first two stagesof the study. Patients identified by these means in whom a diagnosis of dystonia was thought interviewed possible were and examined. Demographic and medical details were obtained and if appropriate the dystonia was classified according to anatomical distribution. Individuals ffilfilling the diagnostic criteria for primary dystonia as describedby the Ad Hoc Committee of the Dystonia Medipal ResearchCouncil (Fahn, 1988) and known to be both exhibiting their condition and resident in the catchment area on the designateddate of January I st, 1996 were included in the study. A history of exposure to neuroleptic drugs or other agents known to be capable of producing dystoniawere causefor exclusion. This gives a good and clear description of the methodology adopted throughout the investigation of all the primary dystonias and has continued in much the same way sincethe publication of the abovepassagesin the autumn of 1998.

41

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART THREE:

METHODOLOGY

CHAPTER 11 Psycho-Social Research A large number of different questionnaireswere usedduring this section of the study. QuestionnaireNo. 30 : Clinical Profile of Dystonia Questionnaire. This instrument was administered once to every consenting participant and was designed,based on the work of Dr Madan Jahanshahiin 1988, and amendedto the ESD format by the author in 1993. It had alreadybeenused by over 300 subjectsin a number of studies ( Jahanshahiand Marsden, 1988a &b and Jahanshahi,1991). A total of 346 subjects completed this questionnaire. It asks the patient a number of factual questions,basedon the following three areas a. Contact with Medical Services b. Clinical Features of the dystonia c. Nature ofyour accommodation QuestionnaireNo. 40 : Torticollis Questionnaire.(attachedto QuestionnaireNo. 30) Again designedand used (as above) by Dr Madan Jahanshahi,a total of 155 of these questionnaires were completed and related to specific questions on torticollis, laterocollis, retrocollis and antecollis,ie :d. Typeand direction of torticollis e. GesteAntagoniste 'Geste Antagoniste' is the term used to describeany sensorytrick used to keep the head in the midline position. It has been known for some time (Patterson AndLittle, 1943) and can be defined as : "7he severity of the abnormal posture and of the involuntary movementsof the head is affected by body position, offell being worse when walking and relieved in the supine position. Patients use 'trick' movementsto keep the head in the midline" (Jahanshahiand Marsden, 1989b). QuestionnaireNo. 50: Living with Dystonja Questionnaire: A PUchological Profile. This questionnaire was administered once during the ESD to every consenting parlicipant. A total of 335 of thesequestionnaireswere completed during the courseof the ESD. It is a combination of four instruments;the first two of which have been used in other torticollis studies (Jahanshahiand Marsden, 1990a and b; Jahanshahi, 1991; Jahanshahiand Marsden, 1992) and had beencompletedby 294 patientsprior to the start of this study.

42

Dystonia -A comprehensiveand longitudinal study in the North East of England

The second two have been used in countless studies and have been validated many times over (seebelow). a. Functional Disability Questionnaire(FDQ) This 27-itern scalewas devisedto assessthe effects of torticollis on activities of daily living. It has been previously shown (Jahanshahiand Marsden, 1990a and b) to have good internal consistency,construct validity and test-retest reliability. Each item is based (not on a0 scored at all affected) to a4 (severely affected). The total scores range from between 0 to 108, with higher scoresindicating higher disability. Basedon previous reliability and validity analyses,3 of the 27 items were sometimeson-kted. b. Body ConceptScale (BCS) Patients rated the concept, 'my body', on 22 sevenpoint semantic differential scales. The internal consistency,construct and concurrent validity and test-retest reliability of the scale have been shown to be acceptable(Jahanshahiand Marsden, 1990a and b). Scoresrange between 21 and 154, with higher scoresindicating a more negativebody concept.Based on previous reliability and validity analyses,someitems can be omitted. c. Beck DepressionInventory (BDI) This 21 item scale measuresdepression. Each item consists of four statements " representin increasing severity of depression,scored0 to 3. Scoresrange from 0 and .a 63, with higher scoresindicating Maher levels of depression(Beck, 1961). 0 C' d. Rosenberg'sSelf-EsteemScale (SES) The 10-items of this scale assessan individual's feelings of self-worth and selfdeprecation (Rosenberg, 1965). The responsecategories constitute a 5-point agreedisagreeformat. Evidence (Warr and Jackson, 1984) suggeststhat the positively and negatively worded items of this scale reflect two independentaspectsof self-esteem. Also, lack of positive self-esteemis reportedly less predictive of depressionthan selfdeprecation (Brown et al, 1986). Therefore, a positive self-esteem and a selfdeprecationscore were derived, eachwith a rangeof 5-20. 1 Questionnaire No. 60-: Impact of Dystonia Questionnaire.

This instrument was administeredonce at the end of the CUA or during the ESD to every participant and is an Acceptance of Illness (AOI) questionnaire. This 8-item scale consistsof statements-relating to the patient's ability to accept the reality of his (or her) illness and to adjust to it. Patients indicate their extent of agreement/ disagreementwith eachitem on a5 point scale. Total scores are derived by summing across and dividing by the number of items. Scores range between I and 5, with higher scoresindicating less acceptanceof illness.The last question measureswhich of four stagesthe patient has currently reached. These stagesare generally acceptedas being Shock, Anger, Despair or Acceptance(Felton et a], 1984).

43

Dystonia -A comprehensiveand longitudinal study in the North East of England

Th.is particular questionnairealso has a final open ended question, which will enable the researcherto determine how successfulthe researchorganisation has been and if there are any lessonsto be learnt for future researchprogrammes, in methodology, presentationor personalattitudes. A total of 338 questionnaireswere completedand returned. QuestionnaireNo. 70 : Primaly Carer'sQuestionnaire. " Too often in the past, socio-medical research has tended to concentrate oil the person to whom the disease or disorder has presented itself and little or no measurementhas takenplace with thefamily or carers of that person . Yhishasbeen considered to be one of the most important areas of research which has been almost totally ignored by the modern social epidemiologist." (Mechanic, 1988) Becauseof the sentimentbehindthe above statement,this instrument was administered once during the ESD to every participant's primary carer in the form of a Carer's Strain Index (CSI). There is spaceat the beginningto indicate the carer's relationship to eachsubjectin question. This 13-item scaleconsistsof statementsrelating to the primary carer's ability to cope with the subjects' illness and how or if they have had to adjust their own life to it. Patientsindicate their extent of agreement/ disagreementwith each item on a4 point scale. Total scores are derived by adding up the individual scores. Scores range between 13 and 54, with higher scoresindicating less strain on the carer (derived from the Caregiver Strain Index: Robinson, 1983). There was an open-endedquestion added at the end to enable the carer to describe how dystoniahas changedtheir life, if at all. A total of 225 of these questionnaires were completed.

44

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART THREE:

METHODOLOGY

CHAPTER12 Socio-EconomicResearch It is quite difficult to describeexactly which questionswere asked in the field, apart from getting a generalfeel of the demographicdata collected durina questionnaireNo ,, 20, which was entitled the 'demographicand dystonic profile' questionnaire. The important questionsaskedgave an insight specificallyto anything which relatedto the patient's current and previous Social Income Group (SIG), their current and previous incomes and questionsrelating specifically to their current job status, their previous job status and their current methods and levels of income. The specific demographicdata asked related specifically to social and economic information, such as :Geographicallocation - Post Code for determiningcounty, district and postal sector. Age - date of birth, confimiing that the Clinical Infonnation Fonn (CIF) was correct. Gender- male or female. There were no specificquestionsregarding ethnicity. Marital status- noted single, married, divorced, separated,cohabiting or widowed. Number and gender of children, including eldest's and youngest's year of birth. Social Income Group - researcher'splacementbasedon occupation and status. Current Income - patient only (family incomewas noted where no income existed). Number, range and amount of any benefits,allowances,pensions,etc. All of the above information was collected and placed on the SPSS file in order to maintain its confidentiality. The rest of the questionsreally related to getting a feel for the person's socio-economic status before the onset of the dystonia and any changes since that time. The whole point being able to discern whether the socio-economic statusof that personhad changedfrom the time of the onset of their dystoniaup to the point of interview. This was generally quite easy as the vast majority of patients were very open and respondedvery well to the reasonswhy these questions were being asked and their answersreflected the truth of the situation, in which they now found themselves. This was much appreciated by the researcher and often reflected the need for further discussionsoutside the current researchdata. The use of the Dystonia Counsellorwas greatly appreciatedhere and severalclients were referred acrossto him as they became formally identified.

45

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART THREE:

METHODOLOGY

CHAPTER13 The Dystonia Nurse Practitioner

Project

The post of Dystonia Nurse Practitioner was first created in mid-1997 by Professor NEke Bames for Nurse John Whitaker, who had been working within the dystonia in it had first in Newcastle the rrýd 1980's. the as clinic nurse since started clinic The idea of the project was to seeif "a properly trained outreach nurse practitioner hospital better is than, that that provided at a as good as, or could provide a service botulinum dystonia toxin. " treatment with requiring outpatient clinkfor people with In doing so, it was to seeif an outreach nurse could administer the drug effectively to as good a standardof efficiency as the doctors were already doing in the clinic. The main motive behind the idea was that the doctors could see that once the patient was be injections little be that there to gained and much could on a standardmode of was doctor, by injections by having than the a rather administered a nurse achieved by home. if injections in Remember that, the the own were given patients' particularly 1997, the numbersof patients had increasedconsiderablyand whereasin the 'old days' the clinic was administeredby a single doctor and a nurse being run once a month, by (to it doctors two three the and was put clinic was run weekly using and nurses now mildly) extremelybusy The first part of this two year research post was training of the nurse in order to administer the Botulinum Toxin therapy without supervision,followed by a proposed 18 month administration of the drug within an arbitrarily selectedgroup of half the doctors by half being the the the the at other of patients administered clinic's patients, ZP clinic in the normal way. Before the processcould start, a very carefully selectedcriteria had to be adopted to ensure complete fairness and independenceregarding the patient selection. The following was adopted by the researchteam of Professor Barnes, John WUtaker and the author and was used in the processto effectively monitor independentlythe work of the nursepractitioner. The Inclusion Criteria were :People attending the movementdisorder clinic at Hunters Moor Regional NeuroRehabilitation Centre with a definite clinical diagnosis of Spasmodic Torticollis, Blepharospasm,Hemi-Facial Spasmor other SegmentalDystonia, Hemi-Dystonia or GeneralisedDystonia. Treatment of dystonia with botulinum toxin injections on at least two preceding occasions,with a clinical needfor such injectionsto continue. Willingness to give fully informed consent to participate in the study by the individuals concerned.

46

Dystonia -A comprehensiveand longitudinal study in the North East of England

The Exclusion Criteria were :" " " " " "

Inability to travel on a regular basis to the outpatient clinic (as would be required as a cornrnitmentover the study period). Pregnancyor child bearing.potential. Psychiatric or other psychological problems that made, in the opinion of the investigators,compliancewith the study protocol unlikely. Previous known allergic susceptibilityto botulinurn toxin. Previous seriousside effects or other reactionto botulinurn toxin. Complex or variable dystonic movementdisorder that required variation in muscles injected with botulinurn or significant variations in other treatments on a clinic by clinic basis.

Having found out which patientswere available,it was then necessaryto decidewhich individual be by in done This to the patient's patients were which group. was using Identification Number as a three digit number being selectedusing random selection procedures. The patient's Clinical ReferenceNumber could have been used but as it was 4 digits, it was agreedthat their ESD referencenumber being 3 digits was much letter be in Having to selectedwhich patientswere which group, a was more efficient. then sent out to eachpatient inviting them to take part in the process. They were then individually contactedand the processexplainedto eachone very carefully. A total of 126 people met the initial criteria and were invited to take part in the for indiNidual determine Because treatments those with there to process. was a need SpasmodicTorticollis, Blepharospasm,Writers' Cramp and GeneralisedDystonia, a , total of 64 people were invited to take part in the Home Group, whereasthere were. only 62 in the Clinic Group.

47

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART THREE:

METHODOLOGY

CHAPTER14 Data Input and Coding Frame The data is input onto a computer in an SPSS(statistical package for social science) file entitled 'phddata.sav'. This file containsall the six years data from 6th May 1993 to 5th May 1999, but it has also beenused on other associatedresearchprojects :ESD = Epidemiological Survey of Dystonia

This containsall the data availableat the end of the input and will be the main file used for this thesis. QUA = Cost Utility Analysis

This file represents all the data collected on subject's No 001 to 231 inclusive. However it is important to note that No's 148,152,159,170,175 and 210 were all non-dystonic CUA controls and have been since removed from the file after the completion of the Cost-Utility Analysiswas completedand published(seepublications) EPD = Epidemiology of Prima! y Dystoni

This was used specifically in the work since published by Dr Philip Duffey and the author and it is vitally important to understandthat only the data which confirmed that if the subjecthad a primary dystoniawas used.All other data was not used at that time DNP = Dystonia Nurse Practitioner

This data related only to those 126 subjectswho Wereselectedas part of the Dystonia Nurse Practitioner researchproject. They are identified by a specific 'C' for Clinic or 'H' for Home in that particular section of the programme. Everyone elsehas a 'N' for cnotin the dnp study' againsttheir file referencein the column marked 'dnp location'. PSR = PUcho-Social Research

This specifically relates only to all the subjectswho have a number from 001 to 550 (whether they agreedto participate or not) plus three other subjects,ie No's 564,594 and 677 - who have since completed all the necessaryforms under this research project. This particular part of the researchis strictly in collaboration with Dr MaIjan Jahanshahiwho, it is hoped, will be publishingfurther results later. IFD

= The Impact of Focal Dystonia on the Working Life of Musicians

This researchproject only related to three selectedcases,ie No's 005,542 and 594, who had been,or were musicians,and had a focal dystonia.

48

Dystonia -A comprehensiveand longitudinal study in the North East of England

SER = Socio-EconomicResearch This particular research project is discussed in detail in this thesis and relates specificallyto all data availableat the time. Patient Numberinizand Status. The patient'sidentification (11D)numberis automaticallydeterrainedby the row number and the column shown asESD is usedfor ranking purposesonly. NB : Six CUA patients have been removed and replaced by ESD patients between 04.12.95 and 04.03.96 - thesewere No's 148,152,159,170,175 and 2 10. The rest of the numberingsystemis madeup as follows -00 1 to 199 inclusive: were active CUA patientscoded A, B or D 200 : is a new ESD patient codedM 201 to 231 inclusive: were externalcontrol CUA patients,re-coded to ESD 232 to 300 inclusive: are new ESD patientscoded A, B or M 301 to 347 inclusive: were (1994) 500 Seriespatientsre-numberedto 300 348 to 937 inclusive: are new ESD patientsnumberedand coded accordingly Syntax Files

Syntax file copies have been enclosedseparatelyin the appendicesand no formulae have beenincluded in this file. The 7 syntaxfiles were run in the following order :phd-geog.sps phd-all.sps phd-dec.sps phd-sf36.sps phd-qual.sps phd-psr.sps phd-env.sps

Recoding 'post codes'into 'county' and 'district'. 21 computationsre Age, DoB, Dates, Differences,etc 12 computationsre defining decimalplacesin the above. 29 computationsre EuroQol and SF36 as per handbook. 3 computationsre qualitative open-questions. 17 computationsre PSR, as per Dr Jahanshahi'sinstructions. 93 comps 'if env = 1, envaa= 0,' etc., & 'if alg = 2, alga = 2,' etc.

SPSS Coding Frame

The SPSS codes (up to No 275) were used in the Cost Utility Analysis (CUA) but have sincebeenamendedfor the ESD, EPD, SEF, PSR and DNP, as required. All variables are entered as Numeric 4.0; missing values None; alignment Right and column width 4, unlessotherwise shown. The value label positions, ic their number, are numberedas per the list of variableson the working file. The file contains 937 rows (subjects) x 842 columns (variables) and therefore has a total of 788,954 potential data sets.

49

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART FOUR: ANALYSIS

OF FINDINGS

CHAPTER 15 Epidemiology, Prevalence and Incidence There were a total of 937 subjectsregisteredin the ESD with 937 separatevariables encodedon the SPSSfile, thus producing 788,954 data sets, at the time of the analysis afler 6th May 1999. The Epidemiology of Primary Dystonia (Duffey et al, 1998) has shown that the crude point prevalenceratios of all the primary dystonias, within the population of the 4 counties of North East England, is 14.28 per 100,000 people, with 95% confidence intervals from 12.8 to 15.75. This breaks down into 12.86 per 100,000, with 95% confidence intervals from 11.45 to 14.25 for Focal Dystonias and 1.42 per 100,000 (95% c.i. 0.95-1.89) for Generalised. This prevalenceshowsthat I in 7,000 people havePrimary Dystonia in the region, but when adding the known and estimated figures for secondarydystonia (Marsden and Quinn, 1990) it goes to prove that dystonia is secondonly to Parkinson's Diseaseas the most prevalent movement disorder, more prevalent than other better known diseasessuch as MS and AIND (MarsdenandFahn, 1998). Whilst the Epidemiology of Primary Dystonias (EPD) restricted itself to the 2.5 million population of the four North Eastern Counties of England, this research had no such boundaries. Socio-Economic research makes no distinction between primary and secondary dystonias, because an abnormal posture or involuntary muscle spasm presents in exactly the same way in both cases. If the subject is unemployed due to their dystonia, it does not matter if it was induced, genetically inherited or idiopathic they are still economically vulnerable.

In more general terms, the concentration on Darlington in 1996 has shown an even more dramatic increasein diagnosisand thereforethe known prevalenceof all forms of dystonia. Currently a total of 42 people display some form of dystonic spasmwithin the pqpulation of 101,766 in the Post CodesDL I, DL2 and DU - this gives a crude prevalencefigure of 41.27 per 100,000population (or I in 2,400) for this town alone. Although prevalence in Durham, Tyne & Wear and Cleveland have approximately similar results, there is a distinct reduction in Northumberland, Cumbria and North Yorkshire. The author believesthat the main explanationfor this is due to the fact that awarenessraising in these specific rural areaswas carried out to a lesser degree of intensity than in the more urban areas.

50

Dystonia -A comprehensiveand longitudinal study in the North East of England

During the researchon the three musicianswith dystonia (Butler and Duffey, 1997), it was established that the prevalence of dystonia within the ranks of professional in musicians Germany was reported as high as I in 500 (Altenmuller, 1997). The reasonfor this may be explainedin that professionalmusicians,almost more than any other occupation, make very fine repetitive movementsof the fingers and hands and that very small dystonic tremors or postures, that are not noticeable nor cause a problem in others, can be devastatingfor them. According to Altenmuller: " Focal limb dystonia of the tipper extremities occurs more frequently in musicians (prevalenceabout 1:500) than in other professions requiring highly skilled movementsof the hand (prevalence about 1:3400). Yhe increased incidence of dystonia in musiciansmay be related to specific qualities of their sensory motor skills; 1) routines for stereotyped movements are rehearsed for extended periods of time with gradually increasing degrees of complexity, 2) via auditory feedback, the motor performance is extremely controllable by both performer and audience, 3) rehearsing andperformance is closely linked to emotion. " This last point is uncomfortably close to suggestingthat dystonia is still linked to a psychogenicdisorder, neverthelessthe significanceof Altenmuller's research is well worth noting. However it is interestingto note :1. The EPD (Duffey et al, 1998) has a proven prevalenceof I in 7,000 (for primary dystoniasonly) 2. Nutt (Nutt et al, 1988) talks of a prevalenceof I in 3,000 in the USA (but based on just 34 people with dystonia) 3. The Darlington study (Butler and Duffey, 1996b) showsthat I in 2,400 of the local population have someform of dystonic spasm. What has been shown by all of the above is that the more researchis undertakenthe more the prevalencefigure increases! The point being that if dystonic musiciansare truly shown to have a prevalenceof I in 500, how much more work is required within the ESD to arrive at an absolutelyvalid figure ? There is no reasonto supposethat musiciansare more prone to dystonia than any other group of people, merely that the very nature of their occupation brings out the dystonia to a greater degree by the very nature of the fine repetitive movements required.

51

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART FOUR: ANALYSIS

OF FINDINGS

CHAPTER16 Diagnoses,previous and current Diagnosis Becausediagnosis plays a major factor in getting sufficient numbers of people with dystonia available for researchpurposes, it was decided in 1992 that an awareness be in run conjunction with the local TDS SHG's. This has raising campaign would itself in a number of seminarsfor medical professionalsand a seriesof seven manifest training videos ýmadeat Hunters Moor in Newcastle, as well as various radio and television broadcastsand newspaperarticles being written over the 5 year period. All of this has helped to raise awarenessof dystonia amongst the medical professionals and the generalpublic which has dramaticallyincreasedthe number of diagnosesmade. The total number of subjectsdiagnosedeachyear to date has seen a steady increase, but there were only 33 subjectsdiagnosedfrom 1937 to 1977, averagingjust 0.83 per year for those 40 years. The averageover the past 10 yearshas been70.5 per year and the averageover the last 5 years has been 95.0 per year or 1.83 per week. However this is only relevant in so far as it shows that the awarenessraising campaignshave beenworking. What is more significant is the number of onsets each year. Reference to the table below shows that the years 1997 and 1996 have less number of onsets than the'12 yearspreviously to 1985. This can be explainedin that dystonia can oflen take several years to develop from the initial onset and many people do not seek medical assistance until the problem is at least a couple of years old. Ignoring the data for the last 2 years,we seethe averagenumberof onsetsfor the 5 yearsfrom 1991 to 1995 as being 53.2 per year. The averageis 44.2 per year for the period from 1986 to 1990 and 19.2 per year for the period from 1981 to 1985. There is no neurological reason to supposethat the incidence will vary by this wide margin over three consecutive 5 year periods, therefore it could be argued that the reasonwhy we see a gradual increasein onset data in this time frame is a direct result of the increasedawarenessin newly diagnosedcases. Therefore the epidemiology has not yet reachedall those people with an onset date over 10 years ago, who either have yet to be diagnosed or who have been diagnosed and yet remain unknown to the epidemiologicalregister. The introduction of Botulinum Toxin (BT) therapy has had a tremendous impact on the rate of diagnosisand has helpedcorrect some of the errors in diagnosisof the past. However regardlessof the reasons,it is entirely feasibleto assumethat a large number of people with dystonia in the region still remain undetectedby the ESD. As to how many,this dependson which 5 year incidenceratio one uses.

52

Dystonia -A comprehensiveand longitudinal study in the North East of England

TABLE Year 1999 1998 1997 1996 1995 1994 1993 1992 1991 1990 1989 1988 1987 1986 1985 1984 1983 1982 1981 1980 1979 1978 1977 1976 1975 1974 1973 1972 1971 1970 1969 1968 1967 1966 1965 1664 1963 1962 1952-1961 1942-1951 1932-1941 1921 -1931 Totals =

1

14. Onset vs Diagnosis in each year.

Onset in the year 1 15 26 39 47 81 72 53 54 69 36 48 39 42 25 24 15 18 21 22 10 25 10 14 18 9 7 6 6 3 2 8 6 4 6 9 6 0 20 12 6 3 937

Diagnosis in the year 21 71 117 87 103 90 96 69 60 49 37 19 12 11 10 5 10 8 9 13 2 5 4 4 2 1 2 4 0 1 2 3 0 0 0 2 1 1 4 1 1 0 937

53

Notes Project end End 5th project year End 4th project year End 3rd 2roject year End 2nd project year End Ist project year Project started 6h May

TDS (NE) formed BT available in the NE

TDS formed

DNIRF founded

Dystonia -A comprehensiveand longitudinal study in the North East of England

The undetected population could be between 720 and 1810 people, assuming an average 7.6 years between onset and diagnosisand an average age of 39.4 years at (both statistics taken from the results of the ESD) and using the Life Tables onset (CSO, 1993) which show male life expectancyat birth to be'72.7 years (78.3 yearsfor females)and at 40 years (the closestto 39.4) male life expectancyis 74.8 years (79.6 years for females). This meansbetween46.5% and 68.6% of the dystonic population remainsundetected. An alternative method of calculating the undetected population would be to extrapolate the various study figures for the entire region. The population of the four northern counties was 2,605,100 (U. K. Office for National Statistics, Estimated ResidentialPopulation) in rnid-1995. The best set of prevalencedata yet known in the region are the Darlington study figures deducedat I in 2,400, which gives a dystonic population for the four northern counties of the North East of 1,085. A total of 779 primary and secondarydystoniasare already known in that geographical area (as of 05.05.99), thus the percentageremainingundetectedis 28.2% or.306 people. The lowest undetectedpercentage,basedon the onset data, is 46.5% and the estimated undetectedpercentage,basedon the epidemiologicaldata, is 50.3%. Although both these sets of figures are relatively close, they indicate the tremendousamount of work still required to do. The referrals to the Bot. Tox. clinics (including previously diagnosedas well as newly diagnosed)has remainedfairly constant since the start of the study at 2.6 per week. This has seen the frequency of the clinics increasefrom once a month, to twice a month, to once a week during this period. The cost of supplying the Botulinum Toxin has also dramatically increasedduring the same period. The Hunters Moor clinic which spentlessthan 150,000 per year on Bot. Tox. at the start now has an annualbill of E510,000for toxin alone (data from fiscal year 1997/98). The diagnosisof individual casesof dystoniahasbeenthe single most difficult pieceof researchto undertake. The previous diagnoseshave often been rough and ready, to say the least. The number of people who have previously been misdiagnosedis very high. The Dystonia Society in its 1993 survey of 705 people indicated that 463 (65.7%) were misdiagnosedat some stage. Our research indicates an even higher figure and with some profound results. In order to determinethe exact nature of this problem, it is necessaryto go back into time and find out exactly what the previous diagnoseswere and seehow this compareswith the presentday diagnoses. In order to do this, we needfirst to establishwhat exactly were the previous diagnoses and how long it took to get a correct diagnosis. 338 people answeredthe question on the reasonsfor any delay in diagnosis. This was principally (according to the opinion of the patients) that their Doctor was unawareof the condition (37.0%). However the secondmost common causewas misdiagnosis(26.4%) and in this case52 people were treated for this misdiagnosedcondition together with a further 14 people who were misdiagnosedand treated for having PD, MS or CP. There was no delay in 13.9% of cases, 10.7% did not seek advice and a further 10.7% had an unknown cause or reason. Finally there were 4 people who were still waiting for an appointment at the time of the researchand anotherpersonwho hadjust gone into remission. 54

Dystonia -A comprehensiveand longitudinal study in the North East of England

The actual diagnosis was carried out in the vast majority of casesby a hospital or privately consulted Dr or Consultant (78.2%). However, of the remainder, a few (6.7%) were diagnosed by their own G.P., mainly as a result of the campaign of awarenessraising conducted during the course of the research in the region, with Ophthalmologists coming next with 4.4%. Self-diagnosiswas the next highest at 3.8%, with SpeechTherapists conýng next for SpasmodicDysphonia (1.5%). The balancewere madeup of 8 different medical professionsdiagnosingthe dystonia, with 2 people being diagnosedthrough one of their own children having the disease. IC ID Another area of concern was the length of time it has taken not only to obtain a correct diagnosis,but also is a few cases(35 people) to be actually told that they have dystonia. The majority are not specifically children, where one can understand (particularly many yearsago) the medicalprofessionnot telling a child what they had. z Nevertheless,this seemsto have appliedevento the presentday. There were 6 people who had never beentold what they actually had, 2 of whom had been diagnosedin 1996,3 in 1994 and one had originally been diagnosedin 1992 but had still not beentold what shehad. Of the remainder,the greatest differencebetween an actual diagnosisand being officially told what the diseasewas called was officially recognised as 18 years. These numbers are small, ie a total of less than 4%, neverthelessit does just show the extent of the prestige with which the medical professionis still held by the generalpublic. The extent of the present diagnosisis quite staggeringwhen one considersthat at the time of the commencementof the researchprogrammethere were lessthan 150 people known to have dystonia within the region. 'Known' meaningwithin the knowledge of the researchers,The Dystonia Society or anyone outside of the immediate family or medical professionalattendingthe particular patient. As can be seenfrom the yearsof diagnosisabove, one can easilyseethe growth and extent"of this phenomenon. A total of 585 people (62.4% of the final total of 937) were diagnosedwithin the 6 years that the researchproject has been running, although the percentage of onset during this period was only 30.0%. This shows the true extent of the research programme's value.

55

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART FOUR: ANALYSIS

OF FINDINGS

CHAPTER 17 Treatments, previous and current Table 15. Different treatments used and various effects recorded. Treatments Bot. Tox. Therapy Drugs / Medication Physiotherapy Acupuncture Osteopathy Hypnotherapy Surgery Psychologist Counselling Chiropractic Yoga Meditation Alexander Technique Aromatherapy Speech Therapy Healing Hands Wearing a collar Homeotherapy Biofeedback Relaxation Technique Reflexology Heat Treatment Manipulation Psychiatry Rheumatology Orthopedic Surgery Cortisone Injections Ophthalmology Phenol Injections Immunology Hydrotherapy Totals

Previous No 149 287 125 43 34 24 40 33 18 12 3 1 2 9 30 8 37 7 2_ 8 6 35 23 43 12 7 4 2 I I I 1007

Current No 493* 232 191 101 98 73 _ 53 49 46 25 20 15 8 5 5 5 4 4 4 3 3 2 1

Effect (%) Better 471(95.5) 4 74(31.9 13( 6.8) 0,99 1 1 ,I.10 5( 51) 5.1) 6( 8.2) 18(34.0) 3( 6.1) 46(100) 3(12.0) 6(30.0) 6(40.0) 4(50.0) 3 (60.0)

Effect (%) Unchanged 20( 4.1) 111(47.8) 164(85.9) 90(89.1) 91(92.9) 66(90.4) 31(58.5) 41(83.7) 21 (84.0) 11 (55.0) 9(60.0) 4(50.0) 2(40.0)

Effect (%) Worse 2( 0.4) 47(20.3) 14( 7.3) 2( 2.0) 1( 1.4) 4( 7.5) 5(10.2) 1( 4.0) 3(15.0)

4(100) -

1440

NB :* There is a section in this category entitled "Too early to define" - there were a total of 80 patients still under this category at the time of closing the file and this numberhas not beenincluded abovenor in the % calculations. 56

Dystonia -A comprehensiveand longitudinal study in the North East of England

Although this research programme has been built up mainly through the current treatment for dystonia, particularly Botulinum Toxin Therapy, nevertheless the previous treatments should be noted and some of the lessons explored. The above chart lists the treatments previously used as describedduring the interview, together with those treatmentsused since, together with their effect. Only 373 people detailed their 1007 previous treatments,averaging2.7 each, however it does show the extent to which patients have tried to obtain some relief from their constant muscle spasms. As can be seen, the far most effective treatment is definitely the Botulinum Toxin therapy. Not only does it score by far the highest, but also its non-effect category is relatively small and the bad-effect category is very small. By far the most effective type of treatmentis Counsellingwith 100% effectiveness- more of this later. Botulinum Toxin Therapy and Side Effects What can also essentiallybe seenis the reduction in other forms of therapy compared to the tremendousincreasein Botulinum Toxin therapy. This is a measurableincrease and this therapy is now currently the first line treatment for dystonýia,although it must be rememberedit does not 'cure' the disease,merely makes it more acceptableor comfortable to live with. As can be seen,not everyonewith dystonia,currently uses this toxin, for a number of different reasons. The total figures currently for its use within our own dystonic population is as shownbelow. Of the current survey population of 937, exactly 22.9% (215 people) have never been injected. A further 10.2% (96) have only beeninjected once with a further 5.2% (49) people having had a 2d injection as part of their current treatment phase. This means that a further 145 people are currently still in the interim treatment stage. Of the remaining 577 (61.7%) people, they currently belong to the largest regional group of dystonia patients and two of the three hospitalscurrently injecting the toxin within the region are currently first and third in supplying toxin in the UK. The way the toxin has been used during the past 6 years has also been monitored and checked. The following chart is not intendedto be a measurementof effectiveness,merely of usage. TABLE Bot. Tox. 0 inj I ini 2 inj -Ljýj4 inj

7 inj 9+ in' Ann als

Before start 529 87 69 43 42 33 40 20 17 57 937

16. Measurement of Botulinum

6.5.93 31.3.94 751 34 36 41 49 20 6 186 937

1.4.94 31.3.95 770 9 37 38 54 23 5 1 167 1 937

1.4.9531.3.96 646 53 48 65 67 39 16 2 I -291 1

937

57

Toxin Usage.

1.4.9631.3.97 537 56 70 113 103 37 13 6 I I 400 1 937

1.4.9731.3.98 546 46 48 89 114 59 30 5

1.4.9831.12.98 491 104 87 95 107 42 10 1

391 937

446

Dystonia -A comprehensiveand longitudinal study in the North East of England

The majority of people had their injections according to where in their body the spasmsactually occurred. Occasionallythe injection was not in the particular point of spasmbut from where the greatest effect would be received. For example, there was one patient who had a few injections in her stomachmusculatureto try and reducethe pain occurring in her abdomen. Of the specific injection sites, 40.0% of injections were in the'neck / shoulder position, 26.1% around the eye muscles, 13.0% specifically in the face musculature,8.2% in the throat, 6.5% in the hand or arm, 4.3% in the leg or foot and 1.7% in the back or abdomen. TABLE

Side Effect Drowsiness Hallucinations Nausea Increasedspasms Felt ill / more pain Tiredness/ Lethargy Depression Double Vision Dry Mouth / Throat Lack musclecontrol. Loss of Weight Dizziness Allergic Reaction Headaches Difficulty Walking Addiction Dry Eyes Constipation Hospitalised Skin Rash Elation IncreasedWeight Memory Loss Dysphagia Ptoris Insomnia Bruising / Bleeding Flu-like Symptoms Coughing Tickling Throat Watery Eyes

Totals=

17. Different side effects

Previous type of side effect Current type of side effect 35 0 35 0 33 8 26 11 25 15 21 3 12 0 9 27 9 4 8 55 7 0 6 1 6 1 5 7 5 3 4 0 3 4 3 0 2 4 2, 2 2 0 2 0 2 1 1 73 1 25 1 0 0 23 0 16 0 7 0 4 0 3 0 2 0 1 0 1 265 301

58

Dystonia -A comprehensiveand longitudinal study in the North East of England

These injections were only noted in full detail up until 1997, after which time the numbersand frequenciesof the cliniis got too busy in order to strictly documentevery patient. However they do give a strong indication of the spread of the injections and furthermore a strict record was maintainedon any particular side-effects. It was noted that these gradually decreasedas the injection teamsbecamemore practised at giving the injections, neverthelessthe type and areaof side effects are interesting, particularly when compared to those side effects experiencedby the same patients previously during their various drug regimes. Some of the side-effectsdo not relate specifically to Bot. Tox. Injections, but they do give an overall impression of the type of side effects experienced. The length of time that these various side-effectswas also noted - in particular the differencein time betweenthe two phases. TABLE

Length of time affected One day A couple of days About a week Over a week A month or over Until treatment stopped Undefined period 2-3 weeks Up to 2 months Totals

18. Length of time affected by all treatments

Previous side effects 5 6 5 2 3 108 40 2 1 172

Current side effects 9 44 35 39 40 1 11 23 12 214

The difference is obvious by comparing the two sets of figures. In the first set, the highest proportion by far (62.8%) is "until the treatment stopped" with the second highest figure being (23.3%) being for "an undefined period". This representsa positive resentmentthat the patient had to go through the sheerhell, at times, of trying to find a treatment that suited them. Although there is a tremendous spread in the current set of figures, one can see straight away the difference. The highest figure is for the period of "a couple of days" and that is only 44 people or 20.6%. The difference that this treatment has had on people with dystonia, in general, has been nothýingshort of miraculous. It has had a tremendouseffect on their Quality of Life as well as improving the overall effect of medicalinterventions,as can be seenabove. Previous treatments

Another way of looking at previous treatmentswas to ask eachpatient to tell us which specialist he or she had actually consulted before they were actually diagnosed as having dystonia. This was carried out towards the end of the researchand a total of 362 participated in this part of the research. Two main questionswere asked; firstly who did you consult before you were finally diagnosed with dystonia and what alternative therapieshad you undertakenbefore you were officially diagnosed? The answerswere quite interestingand are shown below.

59

Dystonia -A comprehensiveand longitudinal study in the North East of England

TABLE 19. Different specialists consulted prior to diagnosis and alternative treatments given. Specialists consulted prior to the diagnosis Neurologist Physiotherapist OrthopaedicSurgeon Osteopath Psychiatrist Psychologist Rheumatologist Unknown Consultant Ophthalmologist SpeechTherapist Neuro-surgeon ENT Surgeon Chiropractor Orthodontic Surgeon Paediatrician PaediatricNeurologist Pain Specialist Haematologist Chiropodist Gynaecologist Aromatherapist Plastic Surgeon Geriatrician Immunologist Totals

No

%

140 114 94 92 88 59 59 48 37 31 28 26 20 5 4 4 3 2 2 1 1 1 1 1 861

16.3 13.2 10.9 10.7 10.2 6.9 6.9 5.6 4.3 3.6 3.3 7.0 2.3 0.6 0.5 0.5 0.3 0.2 0.2 0.1 0.1 0.1 0.1 0.1 100%

Alternative Treatments

No

%

Physiotherapy Acupuncture Osteopathy Hypnosis Counselling Surgery Psychotherapy SpeechTherapist Heat/Shock Treat. Chiropractic Reflexology Traction Yoga Relaxation Tech. Manipulation Faith Healing Experimentaldrug Homeopathy Hydrotherapy Aromatherapy Behave.Therapy

179 100 95 72 52 47 36 34 24 19 12 11 11 10 10 9 8 8 8 7 7

23.6 13.2 12.5 9.4 6.9 6.2 4.7 4.5 3.2 2.5 1.6 1.4 1.4 1.3 1.3 1.2 1.1 1.1 1'.1 0.9 0.9 -

Totals

759

100%

This meansthat 429 people consulted an averageof 2.0 specialistsplus another 1.8 alternativetherapiesbefore getting a correct diagnosis. Medications A comprehensivelist of all medicationstaken by the patients was logged at the time of interview. Thesewere then placedinto various categories,as best as possiblein order to determine if the medication was given for dystonia primarily, to alleviate the conditions of dystonia secondarilyor not for dystonia at all. Of course a number of medicationscould be placed in a number of different slots but this was determinedin conjunction and with the advice of the medical people on the team. The number of medicationsgiven were as shown on the following page.

60

Dystonia -A comprehensiveand longitudinal study in the North East of England

TABLE 20. Different Primary Medications given

Name of medication

Primary medication (n)

Diazepam Benzhexol Baclofen Co-careldopa Clonazepan Tetrabenazine CannabisResin Co-beneldopa Orphenedrine Dantrolene Primidone Selegiline Benztropine Pimozide Thioridazine Trifluperazine Haloperidol Chlorpromazine Levodopa Methixene Lysuride

53 45 24 22 21 11 10 - note an illegal drug 10 9 Sub-Totals 5 3 Benzodiazepines 74 3 2 2 Antimuscarinics 57 2 47 Others 2 I 29 Spasmolytics I I 22 Neuroleptics I I Totals =

229

= 32.3% = 24.9% = 20.5% = 12.7% =

9.6%

229 = 100%

The above drugs are considered the primary ones given to people with dystonia. There are also a number of other medicationswhich may be prescribed which are consideredby someas efficaciousas these. Of the 229 people noted as taking at least one of the above drugs, the vast majority (72.1%) can be seento be taking just the first 5 on the list. The main drug taken is Diazeparnclosely followed by Benzhexol. Although a lot has been talked about the for is it Diazepam, the the given medications most effect of over-prescription of one of the various forms of dystonia. The use of Baclofen has been quite controversial, especiallywith the introduction in the USA in particular, of Intrathecal Baclofen usage. This is where an internal pump is placed,usually to one side of the frontal stomacharea,which feedsBaclofen directly into the spinal cord and it has proved useful, although the trials are still progressing,in limiting severemusclespasmsto do with the trunk itself The following drugs are consideredmore of a secondarynature or, in other words, they are not treating the symptomsbut the effectsof the disorder.

61

Dystonia -A comprehensiveand longitudinal study in the North East of England

TABLE 21. Different Secondary Medications

Name of medication

given.

Given to no. of people as secondary medication

Co-proximal Arnitripyline Co-codamol Carbamazepine Dothiepin Paracetemol Ibuprofen Temezepam Co-dydramol Diclofenac Dihydrocodeine Nitrazepam Fluoxetine Hydrochloride Propranolol Sodium Valproate Hypromellose Clomipramine Phenytoin Lorazeparn Chlordiazepoxide Procyclidine Solpadol Lofepramine Lormetazepam Primidone Chlormethiazole & Clobazam Diflunisal & Dipipanone Fluvoxamine & ln-ýipramine Indomethacin & Naproxen Pergolide & Piroxicam. Remoxipride & Solpadeine Sulpriride & Zopiclone Benorylate & Buprenorphine Dipyridamole & Doxepin ' Feldene Gel & FlurbiProfen Methadone & Naftidrofuryl Parafon & Paroxetine Risperidone & Tiaprofenic Acid Trimipramine Totals =

35 32 24 23 16 14 13 12 12 11 10 9 8 8 8 7 5 5 5 4 4 4 3 3 3 4 4 4 4 4 4 4 2 2 2 2 2 2 1

(2 each) (2 each) (2 each) (2 each) (2 each) (2 each) (2 each) (I each) (I each) (I each) (I each) (I each) (I each)

319 given to 145 patients - averaging 2.2 each

62

Dystonia -A comprehensiveand longitudinal study in the North East of England

There were then a further total of 115 different other drugs given to the patients for other problems not associatedwith their dystonia nor any of the associatedproblems dosages it. if These to the number of noted. are available required as are related

Prescriptions The frequencyof prescriptionwere noted. TABLE 22. Frequency of Prescription.

No. taken

When taken As required (prm) Once a day (od) Twice a day (bd) 3xa day (tds) At night (nocte) 4xa day (qds) Injections (prm) Taken weekly

97 73 51 43 36 30 4 3

Totals

337

As were the numberof methodsof prescription noted. TABLE 23. Different methods of Prescription

PrescribedFree of Charge Patient paid NFIS rates Patient paid privately Totals =

236 174 9 419

63

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART FOUR: ANALYSIS

OF FINDINGS

CHAPTER18 Comorbidity and The Quality of Life Quality of Life was measuredusing the EuroQoI and SF36 measurements.The results SPSS techniques and are shown below, as are the various were analysed using C) Comorbidity measuresadopted. Each will be detailedseparately. Comorbidity Comorbidity was measuredusing the standardisedquestions and can be stated as a definitive measurein the 442 people(47.2%) who responded. The question asked was " What other conditions currently affect you " and the answerswere divided into: 1. Those directly related to dystonia 2. Those indirectly relatedto dystonia 3. Those not related to dystonia. 45 people (4.8% of the entire total) died either before (11), during the study (17) or since completion (17) - this was noted in this section but only 2 peoples'deathswere in any way related (seelater for further information). A number of specific diseaseswere classedas being related to dystonia, or of primary considerationas a specific causeof their dystonia,in a total of 94 (10.0%) people. TABLE

24. Comorbidity

of patients - directly related to dystonia.

19 people had had a Stroke (relatedto dystoniaor not) 13 have Parkinson's' Disease(relatedto dystoniaor not) 12 have Cerebral Palsy (related to dystoniaor not) II haveMultiple Sclerosis (relatedto dystoniaor not) 9 have beendefined as having Tardive Dyskinesia 8 haveBenign Essential Tremor 8 have Spasticity. 3 with an Head Injury 2 with Multiple System Atrophy 2 with Anoxic Brain Damage 6 with each having Chiari Malformation, Gilles de Tourettes Syndrome, Leigh's Disease, Myoclonus, Shy Drager's Syndrome or Pneumococcal Meningitis.

64

Dystonia -A comprehensiveand longitudinal study in the North East of England

A further 16 diseaseswere definedas indirectly relatedto dystonia. These 105 people, ie 11.2% of the total, are listed below. TABLE

25. Comorbidity

of patients - indirectly

related to dystonia.

21 people were officially diagnosedas having Depression 19 were diagnosedas having Cervical Spondylosis 14 had an Anxiety Neurosis 9 had Epilepsy 8 suffered from Insomnia 6 were officially recordedas Drug Addicts 6 had Learning Difficulties 5 had Schizophrenia 4 had a Personality Disorder 3 were Alcoholics 3 were Manic Depressives 2 people had Agrophobia 5 people, eachwith Anorexia Nervosa, Cerebellar Ataxia, Parkinson's' Disease and Post "Psycho-Surgery".

It should be noted that each of these 105 cases were assessedas being indirectly related to dystoniabut there were a further 492 not specifically related to dystoniabut which were recorded to define the 74 other diseaseswhich were present at the same time as dystoniawas also present,of which only the most prevalent are shown below TABLE 26. Comorbidity of patients - not related to dystonia 57 casesof Hypertension were recorded - this represented6.1% 53 casesof Arthritis were also mentioned= 5.7% 52 casesof Thyroid Diseasewere specificallynoted = 5.5% (more of this later) 24 casesof Angina were found, which represents2.6% 21 casesof Asthma, this is equal to 2.2% of the total 19 casesof Ischaemic Heart Disease= 2.0% 19 casesof an Ulcer (peptic or duodenal)= 2.0% 12 casesof Cerebrovascular Disease= 1.3% 10 people have Diabetes = 1.1% 10 people have a Hernia = 1.1% 10 people haveMigraines = 1.1% 10 people have Glaucoma = 1.1% There were a ffirther 169 cases exhibiting the remaining 61 different diseasesor conditions, but these represent less than 1.0% of the total and have therefore been exempted from the list. They however do represent a number of generally more common conditions, such as Anaernia, Diverticular Disease, Lumber Pain, Pheumatoid Arthritis and Valvular Heart Diseasewith 6 caseseach.

65

Dystonia -A comprehensiveand longitudinal study in the North East of England

Maior Illnesses 212 patients have had at least one major illness apart from dystonia in their life. 77 had illness, 33 a third and 16 a fourth. As over two thirds of a second major people the patients were women, it was not surprisingthat the major illness was of the female variety. However, it was also interesting that other major operationswere to do with the neck / leg hand the arm or or eyelid surgery. These are listed below as the 20 most or diseases illnesses. or prolific TABLE 27. Other Major Illnesses.

Type of illness

Frequency

Hysterectomy RTA Growth removed Stroke Neck - Operation Cancer- unspecified Leg / Arm / Hand Operation Eyelid Surgery Heart attack CardiovascularDisease Fractures- Bones Appendicitis / ectomy Brain Tumour / Operation Gall stones/ Bladder Thyroidectomy Arthritis Unspecified Operation Hiatus Hernia TB Ulcers 53 Other diseases(averaging2.1) Totals

29 28 15 14 14 13 13 10 10 10 9 9 9 8 7 7 7 6 5 5 111 339

66

Dystonia -A comprehensiveand longitudinal study,in the North East of England

Movements 237 people registered345 types of movementaveraging1.46 each. TABLE 28. Types of Different Movements Type of movement

Frequency

Slow pulling / turning Slight twitching Quickjerking Regular trembling Nfixture of I to 4 above Other various movements

118 72 68 57 8 22

34.2% 20.9% 19.7% 16.5% 2.3)% 6.4%

----------- -------------345 100%

Totals

Of the various other movements,the eyýlids shut = 5, occasional trembling = 5, rhythmical / continuous movements= 4, crampsor cramping = 3, stiffhess= 2, voice whispers= 1, suddenjerks (once) =I and Action Induced/ Task Specific = 1. The severity of these movementswas measuredas well as the degree of control and the pain levels as below. The first question asked was " Pleaseindicate how severe you think your dystonia is at present(comparedto others with dystonia that you have seenor comparedto how it hasbeenin the past) by circling the appropriate numberof the scalebelow :" and the resultsare :TABLE 29. Severity of Movement. Severity

Frequency

Not severeat all 1 2 3 4 5 6 7 8 9 Very severe Totals =

%

16 21 28 51 48 67 28 21 26 9 15

4.8% 6.4% 8.5% 15.5% 14.5% 20.3% 8.5% 6.4% 7.9% 2.7% 4.5%

330

100%

67

Dystonia -A comprehensiveand longitudinal study in the North East of England

The secondquestion asked was " Pleaseindicate the degree of control you presently have over your muscle spasmsby circling the appropriatenumber on the scalebelov.?' and the results are :TABLE 30. Degree of control over muscle spasms.

Degree of control No control at all 1 2 3 4 5 6 7 8 9 Complete control

Frequency

%

52 36 26 34 21 50 27 29 24 17 13

15.8% 10.9% 7.9% 10.3% 6.4% 15.2% 8.2% 8.8% 7.3% 5.2% 4.0%

---------------- --------100% Total 329

The next questionwas " Pleaseindicate the degreeof control you presentl have over C, the involuntary movement / abnormal postures of your affected body part(s), by circling the appropriatenumberof the scale:" and the results are :TABLE 31. Degree of control over involuntary

Degree of control No control at all 1 2 3 4 5 6 7 8 9 Completecontrol

movements / abnormal postures.

%

Frequency 43 33 28 28 25 32 27 22 21 10 19

14.9% 11.5% 9.7% 9.7% 8.7% 11.1% 9.4% 7.6% 7.3% 3.5% 6.6%

--------------- ---------100% Total 288

68

Dystonia -A comprehensiveand longitudinal study in the North East of England

Pain levels

198 people indicatedwhere the levels of pain were and they were measuredbasedon a standard level in 14 different parts of the body. A total of 5 different parts of the body were available to be covered individually as follows. Remember this is to indicate where actual pain levelsare located, not which musclesare affected. TABLE 32. Where is the greatest pain ?

Where is the pain

1" No

Neck muscles Shoulder muscles Arm muscles Back muscles Leg muscles Head Hand muscles Foot muscles Trunk muscles Jaw muscles Eye muscles Face muscles Mouth muscles Throat / Larynx Totals =

2d No

3d No

106 12 12 6 10 12 5 4 6 5 10 10 0 1

14 46 12 15 12 16 6 5 3 2 0 2 3 0

4 11 18 8 6 5 4 5 2 2 0 1 0 1

4 3 1 6 5 1 4 3 2 2 0 0 0 0

0 1 2 3 5 1 0 2 3 0 0 0 0 0

128 73 45 38 38 35 19 19 16 11 10 13 3 2

199

136

67

31

17

450

4h No

5h No

Total

This gives a very good picture of the entire body and where in particular the greatest pain resides. As we can seethe greatestnumberof people have pain in the neck, then shoulder and arm. This is mostly becausethe greatestnumber of people are affected with torticollis. However this is not the only area where pain residesas can be seen. There was a question about how frequent this pain was, the answers were 'often' (44.9%), 'continuously' (40.3%) and 'infrequently' only 14.8%. Which meansthat out of 196,85.2% or 167 of them have pain either continuouslyor often. Spontaneousimprovement One of the questionsmost oflen asked of Doctors is "wifl this go away on its own". Therefore we asked it of the patients and the answerswere very interesting as 63 people said Yes, they had had some form of spontaneousimprovement. This was 18.1% of the 349 who answeredthe question so it appearsthere should be about a 20% chanceof someform of remissionduring the lifetime of the disease.

69

Dystonia -A comprehensiveand longitudinal study in the North East of England

We gave each person answeringtwo opportunities to give us the year it started, how long it lasted and if it was partial or complete remissionwhile it lasted. The answers were interesting as firstly of the 63 people who responded, the years of the improvementwere a very wide spreadstarting in 1954up until 1996, with the meanin mid-1990. Secondlythere were 19 people who had had a secondperiod of remission, it was much more concise from 1981 to 1996 and the mean was slightly earlier in 1989. The first time 56.5% said it was partial, the remaindersaid it was complete and the secondtime 57.9% said it was partial and the rest said it was a complete remission. The answersare tabulatedbelow :TABLE 33. Length of time of Spontaneous Remission. How long it lasted

V time

A few days only Several weeks 4 weeks or more 6-8 weeks 2 months 3 months 6 months to a year Over a year 2-6 years 8 years 15 - 27 years Still in remission

9 11 4 2 1 1 6 1 9 2 2 15

Total

-------------- ------------------ ---------------- -----------63 19 82 100%

2

nd

4 3 3 1 1 1 2 1 1 2

time

Total

%

13 14 4 '5 2 2 7 3 9 3 3 17

15.9 17.1 4.9 6.1 2.4 2.4 8.5 3.7 10.9 3.7 3.7 20.7

As can be seenabove, apart from those 17 people who were still in remission at the point we completedtheir study, there were a large number of people (43.9%) who had had a remissionfrom a few daysup to 4 years. What we needto know is what caused this remission. Unfortunately in every caseit was different and there seemsto be no connecting factor, except as one person, still in remission, stated " Don't question it, just acceptit and pray it continues."

Qualityof Life Results The overall results generally compliment those already published under several different sources, therefore the results are not detailed below, although some conclusions can be drawn later. Even though the previously published results only relate to the data collected on the first 239 participants, the final results relate specifically to all the 400+ people who completed all the EuroQoI and SF36 forms detailed, however the methodology has been published previously and the overall results do not differ at all from those previously published only in the detail of numbers. For thesereasons,no data is shown here.

70

Dystonia -A comprehensiveand longitudinal study in the North East of England

PART FOUR: ANALYSIS

OF FINDINGS

CHAPTER 19 Genetics Although geneticswas never originally envisagedas being part of this researchproject; have it first moved on since was started. At the recent International events Symposiumheld in Miami, Florida in October 1996, a vast amount of new data was brought out regardingthis particular subject. At that point in time, a total of sevennew genenomenclatureshad been discoveredall relating to dystonia. Since then, as discussedin Chapter 4, a total of 12 genes are thought to be responsible for different types of dystonia throughout the world. Therefore it was decidedquite late on during this researchto attempt to discover how many people within the region had proof (documented or otherwise) of a genetic ancestryor at minimum a suspicion. Therefore most people have beenaskedthe question: " Are you aware of anyoneelse in your family with any form of musclespasm?" The results are quite interestingand are given herewith. A total of 556 people (59.3%) answered this question, in one form or another. A total of 210 people (37.8% of those responding) answeredin the 346 as the number who thought negative with "no definabletrigger". Thus leaving 4D 0 there might have a "trigger" for their dystonia. Although this is discussedin detail disease". familial "a 47 total of people answeredpositively regarding elsewhere,a However a total of 173 people (28.7%) answered'Yes' to the question :" Do you have any family memberswith dystonia?" 423 (70.3%) answeredNo and a further 6 answeredUnknown - as they were adopted. Adding in the people who had more than follows different 265 total as -a single relation affected, a of relationswere affected TABLE

34. Relationship to others affected

53 had a sister affectedin someway 45 had a mother affected 44 had a maternal relation affected 43 had a paternal relation affected 33 had a father affected 18 had a brother affected 14 had a daughter affected 10 had a son affected 4 had a nephew or niece affectedand I had his wife affected(very unusualto havea married couple separatelyaffected) 265 people had a relation affected in some way

71

Dystonia -A comprehensiveand longitudinal study in the North East of England

This however did not-show the whole picture as a number of people did not have a confirmed or diagnoseddystonia,as follows :TABLE

35. Type of spasm reported in the allegedly affected people.

56 people had a focal dystonia 39 people had generalised dystonia 2 people had segmental dystonia I personhad a hemi-dystonia

A total of 98 people had a definite 'diagnosed' dystonic spasm

71 people had an undiagnosed spasm 63 people had another neurological disorder 33 people had undefined spasms 265 different people with different types of spasms

Therefore the only proven (at this time) number of people with a known dystonic family pattern was 98 and tl-fisfigure is basedon known and provable diagnosesand relates to 10.5% of the known dystonic population. It is however very likely that a figure approaching over 200 is more realistic. This relates to 16.6% of those who were able to answerthe question. This figure is currently in excessof current known patterns of familial dystonia and is an area that should be explored and expanded further. In particular, it would be very interesting to examine the 71 people with an undiagnosedspasmand the 63 people who were related to the patient with dystonia but who had another neurological disorder. This might well pick up a number of other dystonicswho had beenpreviously misdiagnosedand often treated aswefl.

72

Dystonia -A comprehensiveand longitudinal study-in the North East of England

PART FOUR: ANALYSIS

OF FINDINGS

CHAPTER20 Social Implications Social and Economic Groupings

Social class can be defined in a number of different ways using a number of different A classifications. review of the common classificationsof occupationsin social classes in in Britain shows that only one meetsthe requirementsof the empirical research used current researchinto dystonia. " Most commercial, social, advertising and consumer research enterprises use the social grading of occupations, originating from the Institute of Practitioners in Advertising ie, Marketing Research : MR. " (Reid, 1989). However the main reasonthat this classificationhas been used in this researchproject is that it is the only one in common usage that has the non-employed category of retired, on disability benefit, income support and other forms of allowance. TABLE 36. Social and Economic Group (SEG) Categories. A

Professional - defined as successful business persons (eg, self-employed manager / executive of large enterprise), higher professionals (eg, bishop, surgeon, medical specialist,barrister, accountant), senior civil servants(above Principal) and local governmentofficers (chief executive,treasurer,town clerk)

B

Managerial- defined as senior, but not the very top, people in the samearea of employmentas categoryA.

CI

White Collar Workers - defined as small trades people, non-manual, routine administrative,supervisoryand clerical.

C2:

Blue Collar Workers - defined as skilled manual workers, at the top of their trade or skill.

D

Serni-skilled and unskilled workers - defined.as all those people currently in work but not previously categorised.

E

Unwaged - defined as those at the lowest levels of subsistence,including the retired, those on social security becauseof sickness or unemployment, and students.

By using these social gradings of occupations,this researchhas been able to give an insight into a number of different social and econon& factors resulting in a large numberof people with dystorýafalling into the lowest category, ie, being unemployed, prematurelyretired, on incapacitybenefit or income support. However this group also includesa few wealthy retired folk with large occupationalpensionswhich were noted.

73

Dystonia -A comprehensiveand longitudinal study in the North East of England

The following table shows the SEG of the study population and demonstratesvery his (HoH) SEG Household her Head that though the the the at or even of of clearly highestlevel is roughly the sameas the National Average, there was greater weighting in SEG C, amongstthe patientsat their highestlevel. This is due to the fact that 66.1% of the study population are female (confirming double dystonia the number of women to that tends to affect roughly studies previous is 'White female Collar ) that the as categorised majority of employment often and men Worker' or with 'Clerical Status. The following table also shows there is a movementdownwards in patient SEG status. Every category shows a reduction over time, &om QuestionnaireNo 20 at the start of the study to QuestionnaireNo 25 at the end, except 70.2% of the study population is increased in lowest (ie Unwaged) life Eth. the and and economic group social start to 72.8%. All correlations across the table by Chi-Square Test come out as Iýghly fit' 'goodness Chi-Square These tests against the national of results are significant. distribution. average TABLE 37. Social and Economic Groupings (1996) Social Economic Group A Professional B Managerial C, White Collar C2: Blue Collar D: Semi-skilled /Unskilled E: Unwaged, etc. Deceased

n Chi-Square D. F. Significance p

Q're 20 1st SEG 0.7% 3.1% 14.6% 4.1% 5.2% 70.2% 2.2%

460 1214.82 6 1 44 1'4

p 0 0 ý4

U)

(1)

.rq r.

F.:

0 4-) 4-4 Ea rj

. ri P 0

>1la)

En a) rc$

rd 4-4 0

H

>

as 4 4-) fTj 9

fd . 1-1-ri

4J r, .. (1)

Ei En

a) p

4J a)

fd ý 4J Ea :1 F-:

r-I 09

0V 4J U)

Old

>

fo 4-4 40 H

ý4 4J 13) 44 fý -, q

(o r-I

>1 V) rc; :J rý: Ei

fa

a) >4 4 31

4

0

M 4J

ýq

. -I ., 1

ý-: 0 4-) ri

p

tP

(D

P4 0

:j F;

M

tp (a p 0)

(d P4

W0 >1

>1 0 PV

> a) p a) fd

Ei

0

(1) 0)

(D ýq 4-4 m (a 0

X: m id

0) , U) r-I (1) P4

rA (Z Fo 09

cc

4-) 0 m -H

>1 z

44 4-J

4

Q) 4-4 rn 0

4-J fd 0 0 -rq >

a)

a)

4J r. 0

4-4 0

4J

r-I la4

-ri Fr-I -H a)

0)

4 -r-i 0 r-i () -1 4 ý4

a) ýq

0 4-) 4J >1

D. r-I

qj ýQ ý4

a) 4-) >1 ýQ lz 0

a) Ic: 9 4J 0 0

ýO 4-) rý %:: . rl a) CO a) (o E/) P I 4J P4 rd

'0

U)

fo

co

Q)

-, i

-, 1

4H

Z 0 4-J

rý 0 V

4-

>1

>1

"A

ul

La

a)

H 0

fa

0)

0) 4-4 $4 0

4

P:

0 04 r-I a) (1)H (13

00

r-

>1 El)

0 t3l

4P

4-J 0

4-3 r0)

a)

4

r:: Pw

0 >,

1::

Ul 0

0 r-i 0)

rZI (D z

a) (a 41 4J

m t3l 0 P*

a)

0

4-) a) W

H a) > M 4

Lo ri 4-4

3: N 0

: F: Cl) 0

(a

rA Q) 4J >0 (d (1) 4 44 4-1 H (1) "It 0

-

14 En MP

r=; En

0 U

-ri

C.-r-I 4J M

H

Pi

0

:: ) 0

-1 U) 0p a) -r-I 4-) 104

-r-i

E-1

0

>1 4

r.

0 rq V >1 Ell r-I a) r-, 0

-rq 44 FZ4 0

(U 4-) $4 ýl FC4 0

-ri

U)

r-

>1 0 z RS ý4 Q) a) I::: P 4j a) -r-I 4 (1) 4-)

r.

En

0 (n (d

Ell

H

ý4 H

F-I

0

EF)

Ff-= 0 a) () >

U) H

-ri

E-4

0

0

>

-

>4

>1

ro

rj

0 -r-I >i 4-3 td

4-4 rd

rc% rcl

0 0

4-4

e ýQ 0 as ý4 10 4-4

Ef)

E-1

4-1-H

tp--i F-: 44 --i 4J a)

0

z o H

0) 41

r-

P A4

z

U)

::s al 0

H0 04

cn

H

D:

0

m

00 0 >1

4-) 1::

H

4J r.

F- :I

4-)

U) fu

D. U

-4 ý-: 4-4 fd HH

fu

z

a)

as

F;

a) O'C',

E-1

Z

U)

-ri

(15

-P

-

-,Zj

0

to

0

9 -rA 4-3

:J 4J

>1 Z

(d

a) r-4

4-4 4-)

--f >1 p

P4

m -lq >

r-

P

4-) rq r-f (d a)

p

44

::% , 4-) Ul

44

U, PL1

10. P a)

th 0

0) >1 ZP

-r-I p

0

a)F0 rl

7ý 4J H

0.

m

(2)

>

En

>1

ro

>1

44

>-t

rd

-ri

(1)

a)

. 1i p :3: 0

(a

(1)

IDI Ef)

0

rc% Ea

J-z .0 4-)

4-3

0 4-)

co rcl

rz: as

a) ýQ

rc$

ý9

P4

F-I >4

QUESTIONNAIRE

FOR

"

YOUR PRIMARY

CARER

"

form is to be completed by the person This who is most involved in looking (or who lives the person dystonia af ter with) with ). All (the "primary be treated answers will so-called carer" in the utmost form should be placed, confidence and this after into directly an envelope marked C. S. I. completion, (and overleaf) have been Below of statements, which are a list to live who have with people someone who has made by other dystonia. that You may feel some of the statements also apply do not. to you, whilst others indicate how much you Please these by ticking statements, to how you have felt according

agree with or disagree box. the appropriate over the past inonth.

Office

Questions 00)

Today's

Date

01)

What is your relationship the person dystonia with

is

Your

.............

someone I feel

?

03)

04)

05) 06)

07)

My sleep

It

It

It

is

is

is

There family

is

170

II

Agree

Disagree

+--+

+--+

+--+

Strongly Disagree

who that

I I I I

disturbed

inconvenient

a physical

strain

confining have had to adjustments

1 have had my personal

number

....................

+--+

02)

code

is

to

Strongly Agree Living with has dystonia,

each of Answer

be

to change plans

II

I I

+--ý

+--+

+--+

+--+

+--+

+--+

+--+

II

+--+

II

+---+

II

+--+

+--+

+--+

+--+

+--+

+--+

I I I I

II

II I I

+--+

+--+

+--+

+--+

+--+

+--+

+--+

+--+

II

+--+

II

+--+

II

+--+

+--+

+---+

+--+

+--+

+--+

+--+

II

+--+

+---+

+--+

+--+

+--+

+--+

II

+--+

IIII

+-.

-+

+--+

IIII

+--+

+--+

I I II II P. T. O.

(cont)

Office Strongly Agree

08)

Agree

Disagree

Strongly Disagree

have been other There demands on my time

+--+

II

+--+

09)

10)

1 have had to make emotional adjustments Some of behaviour to me

his is

I have had adjustments 12)

is It strain

/

+---+

+--+

IIII

her upsetting

+---+

+--+

+---+

+--+

+--.

+--+

+--+

+---+

+--+

+--+

+--+

+---+

+---+

+--+

+--.

+--+

II

+--+

II

+---+

+--+

+--+

+--+

+---+

+--+

+--+

+---+

II

+--+

+--+

-+

+--+

+--+

+--+

+--+

IIII

+--+

+--+

+--+

IIII

1 am completely overwhelmed

+-.

+--+

14)

15)

is distressing It (name of person) from his changed former self.

II

+--+

+--+

13)

I I

+--+

+--+

IIII

a financial

+--+

+---+

IIII

to make at work

+--+

I I

-+

I I

IIII

+--+

+

II

that has / her

+-. --+

II

+--+

II

II

II

+--+

II

+--+

If you would like to write how you a statement about feel that dystonia has changed in relation life your to the person dystonia, feel free to do so here with +--+

+--+

+--+

+--+

+--+

................................................... Remember

:

everything

you

+--+

say

is

treated

in

confidence. Page

2

QUESTIONNAIRE

ON EXTERNAL

FACTORS

AFFECTING

DYSTONIA

is information this to The purpose of questionnaire obtain f actors dystonic environmental which might af f ect your about in informed the have Several people study spasms. us that f actors the external af f ect either severity certain of their increase levels. As you know yourself or some days pain spasms Why ? Is there ? a common thread are bad. are good and others to We are now able compare a large number if is dystonias different there and to see factors. The between a number of different if help will us to understand questionnaire to any particular common features phenomenon.

Questions 00)

Today's

General 01)

02)

write is

Date

Your

.............

Ouestions

YES,

please

describe

circle

Office

......

code number

Please

is

180

1

answer

in your life pattern, illnesses, etc. of that already aware, illness ? to your

been any changes Have there from major apart operations, is the research which study you think may have contributed

If

answers

of people with any correlation this results of there are any

these,

YES

NO

YES

NO

YES

NO

house?

YES

NO

?

YES

NO

YES

NO

dates

with

................................................. ................................................. ................................................. 03)

Do you

04)

Are

05)

Do you

06)

Are

07)

If

you

you YES,

currently affected have

gas

affected please

smoke by

other's

certain

describe

the

?

smoking in

appliances by

?

regularly

your

sounds type

of

sound(s)

................................................. 08)

Are

09)

If

you YES,

affected please

by

bright

describe

.................................................

light/sunlight how it

affects

you:

-----II -----Page

I

lo)

Is

your

drinking

Tick

water spring Well mains Filtered Bottled

11)

Is

your

The following a) Allergic c) Food and

mains

sections reactions Drink

........... ........... ........... ........... ...........

Fluoridated Soft Hard Softened know Don't

water

Of f ice

which

+----+

II

+-----+

........... ........... ........... ........... ...........

+----+

+----+

into divided :b) Exposure to chemicals d) Environmental substances

are

like We would the next two pages, you to complete but if you do not think by any 9f the you are affected this above, you can finish Page 4. questionnaire after in case any of it Read on, just 1 makes you think 12)

Please down any other write the above) which you think

factors affects

(apart from dystonia your

? +----+

II II

+----+

+----+

+----+

a)

Allergic

reactions +----+

13)

Have

you

ever

had

any

allergic

YES

problems?

NO +----+

14)

If

YES,

please

Puffy

tick

the

type(s)

shown Asthma Migraines Persistent fatigue Hay Fever Arthritis Hyperactivity Rhinitis Mouth Ulcers Stomach Ulcers Wind / Bloating Eczema / hands / face ankles / Hives Uticaria Diarrhoea (describe) other

below ........... ........... ........... ........... ........... ........... ........... ........... ........... ........... ........... ........... ........... ........... ...........

+----+

+----+

+----+

+----+

Page

2

15)

b)

Have any close blood relatives any of the allergic problems Chemical Have

17)

If

18)

Repeated

19)

Was it

20)

Exposure

21)

Other

22)

23)

24)

you

YES,

Food

YES

NO

chemicals?

YES

NO

YES

NO

YES

NO

YES

NO

?

YES

NO

?

YES

NO

------

Exposure

16)

c)

ever had shown ?

been

ever it

was

during

exposure in

exposed crop

fumes

(describe)

pollution

accident

from

?

straying

industrial

to

an industrial to

to

?

a chemical

fire

.....................

and-Drink

Do you think that fluctuate your symptoms as a result of the foods you eat or the beverages ? you drink

-----YES

If YES, are there that any foods you know make you ill now, made you ill as a child ? or make your spasms worse If YES, please which of them If

the following you by circling

check affects

and

-----YES

/

Fish

Cereals

Dairy

Products

NO

indicate the numbe r

you are not affected 0 at all, circle If you are only 1 mildly affected, circle If you are moderately 2 affected, circle If you are severely affected, circle

Meat

NO

3

a. b. c. d. e.

Pork Beef Red meat Plaice Other ........

0 0 0 0 0

12 12 12 12 12

3 3 3 3 3

f. g. h. i. j.

Wheat Rice Corn Oats Other

........

0 0 0 0 0

12 12 12 12 12

3 3 3 3 3

k. 1. m. n. 0.

Milk Egg Cheese Yoghurt Other ........

0 0 0 0 0

12 12 12 12 12

3 3 3 3 3 Page

3

Liquids

/

Fruit

Other

Foods

25)

Have

26)

Are there frequently, you feel

27)

Vegetables

you

ever

been

P. Coffee

0

1

2

3

q. r.

Tea Other

0 0

1 1

2 2

3 3

S. t. u.

Citrus Potato Other

........

0 0 0

1 1 1

2 2 2

3 3 3

V. x. y. Z.

Yeast Chocolate Sugar Other ........

0 0 0 0

1 1 1 1

2 2 2 2

3 3 3 3

........ Fruit

on an exclusion

any foods or drinks you for crave and actually better after consumption

NO

. ...

........................................

.....

. ...

and

tea

29)

How many

cups

of

coffee

30)

Does decaffeinated to any difference

coffee or tea your spasms ?

Do wines, spirits in any way ?

or

If

YES,

describe

do you

drink

of

34)

YES

.....

name them

cups

33)

------

........................................

please

How many

32)

have make ?

NO

have:

YES,

28)

31)

YES

they

If

the

diet?

beer

in

drunk

affect

affect

a day a day

?

....

. ...

....

. ... ------

make YES

affect

NO ------

you YES

how they

NO

you

........................................

.....

. ...

........................................

.....

. ...

Are you affected in drinks, fizzy If

YES,

describe

by Colas or any way ? how they

------

other

affect

Of f ice ------

YES

NO

you

........................................

.....

. ...

........................................

.....

. ...

THANK YOU FOR COMPLETING THIS QUESTIONNAIRE. THE NEXT 3 PAGES NEED ONLY BE COMPLETED IF YOU THINK YOU OR YOUR SPASMS ARE AFFECTED BY OTHER ENVIRONMENTAL SUBSTANCES

Page

4

d.

other

Below extent circling If

environmental

are to

Of f ice

substances

a list of substances. which you are affected the appropriate number,

indicate Please by any of them, as follows :-

the by

you are not affected 0 at all, circle If you are only 1 mildly affected, circle If you are moderately 2 affected, circle If you are severely affected, circle

NATURAL

3

INHALANTS

35)

Pollens

aa. ab. ac. ad. ae.

New mown grass Trees Long GrassHay Other pollens

0 0 0 0 0

1 1 1 1 1

2 2 2 2 2

3 3 3 3 3

36)

Moulds

af. ag. ah. ai. aj.

Damp humid Old houses Fungus Moulds Dust

0 0 0 0 0

1 1 1 1 1

2 2 2 2 2

3 3 3 3 3

37)

Animals

ak. al. am. an. ao. ap.

Dogs Cats Horses Rodents Birds Feathers

0 0 0 0 0 0

1 1 1 1 1 1

2 2 2 2 2 2

3 3 3 3 3 3

38)

Insects

aq. ar. as. at.

Bee stings Wasp stings dust House Mites

0 0 0 0

1 1 1 1

2 2 2 2

3 3 3 3

39)

Plants

au. av. aw. ax. ay. az.

Odour of pines Pine products Houseplants Manure Silage Rotting vegetation

0 0 0 0 0 0

1 1 1 1 1 1

2 2 2 2 2 2

3 3 3 3 3 3

ba. bb. bc. bd. be. bf. bg.

Degreasers Deodorants Hairsprays Polishes Insecticides Pesticides freshener Air

0 0 0 0 0 0 0

1 1 1 1 1 1 1

2 2 2 2 2 2 2

3 3 3 3 3 3 3

CHEMICALS 40) Aerosols

days

liii' Li liii' Page

5

If

0 you are not affected at all, circle If you ar e only 1 mildly affected, circle If yo u are moderately 2 affected, circle If you are severely d, circle affecte

Of f ice 3 +----+

41)

Cosmetics

bh. bi. bj. bk. bl. bm. bn.

After-shave Creams Deodorants Perfume Powder Make-up Shampoo

0 0 0 0 0 0 0

1 1 1 1 1 1 1

2 2 2 2 2 2 2

3 3 3 3 3 3 3

42)

Flooring

bo. bp. bq. br. bs.

New carpets Linoleum tiles Floor Sealer Adhesive

0 0 0 0 0

1 1 1 1 1

2 2 2 2 2

3 3 3 3 3

43)

Foam Rubber

bt. bu. bv. bw.

backing Carpet Cushion Upholstery Padding

0 0 0 0

1 1 1 1

2 2 2 2

3 3 3 3

44)

Cleaners

bx. by. bz. ca. cb. cd. cc.

Ammonia Bleaches Detergents Liquid polishers Silver polish Furniture polish Carpet shampoo

0 0 0 0 0 0 0

1 1 1 1 1 1 1

2 2 2 2 2 2 2

3 3 3 3 3 3 3

45)

Fuels

cd. ce. cf. cg. ch. ci. cj. ck.

Heating oil Gas Paraffin Calor/Butane Coal Fire Charcoal tar Burning Burning rubber

0 0 0 0 0 0 0 0

1 1 1 1 1 1 1 1

2 2 2 2 2 2 2 2

3 3 3 3 3 3 3 3

46)

Motoring

cl. cm. cn. Co. cp.

fumes Petrol oil fumes Diesel Upholstery fumes Exhaust

0 0 0 0 0

1 1 1 1 1

2 2 2 2 2

3 3 3 3 3

47)

Paints

cq. cr. cs. ct. cu.. cv.

New paint Paint stripper Turpentine Varnish Oils Fixative

0 0 0 0 0 0

1 1 1 1 1 1

2 2 2 2 2 2

3 3 3 3 3 3

liii" Li

11 Page

6

If

48)

you are not affected 0 at all, circle If you are only 1 mildly affected, circle If you are moderately 2 affected, cir cle If you are severely affected, circle

Tobacco

cw. cx. cy. cz.

Cigarettes cigars Pipe Cigarette

Smoke

Office 3

0 0 0 0

1 1 1 1

2 2 2 2

3 3 3 3

49)

Solvents

da. db. dc. dd. de. df. dg. dh.

fuel Lighter Carbon Tetrachloride Glues Newsprint White Spirit Dry Cleaning Fluid Methylated Spirits Surgical Spirit

0 0 0 0 0 0 0 0

1 1 1 1 1 1 1 1

2 2 2 2 2 2 2 2

3 3 3 3 3 3 3 3

50)

Others

di. dj. dk. dl. dm. dn. do. dp. dq.

Chlorine Bitumen Tar Asphalt Weedkillers Mothballs Disinfectant Swimming Pools Food Additives Pesticide Residues in foods in Water Pollutants Waxes (on some fruit and vegetables) Tartrazine Phenolic Resin in cans Formaldehyde

0 0 0 0 0 0 0 0

1 1 1 1 1 1 1 1

2 2 2 2 2 2 2 2

3 3 3 3 3 3 3 3

0 0

1 1

2 2

3 3

0 0

1 1

2 2

3 3

0 0

1 1

2 2

3 3

dr. ds. dt. du. dv. 51)

Smells given

-----of

by

dw. dx. dy. dz.

Plastics Coated Paper New Fabric Others ............

The above list is not long although very If there any other elements which affect the appropriate add them below and circle 52)

Any

other

substance

0 0 0 0

1 1 1 1

2 2 2 2

comprehensi you, please number

3 3 3 3 ve.

.............

0

1

2

3

.............

0

1

2

3

..............

0

1

2

3 Page

7

(cont) 52)

53)

Any

other

Of f ice substance

.............

0

1

2

3

0

1

2

3

0

1

2

3

0

1

2

3

.............

0123

.............

0123

Any there that any other comments you may wish to this write about factors whQle area of external which you think might affect either your dystonic threshold levels ? Why, for spasms or your pain do you think that example, some times are better than ? Do you have a theory this ? others about +----+

+----+

+----+

+-----+ +----+

+----+

II +-----+

+----+ +----+

+-----+

II

+----+

+-----ý

+-----+

Page

8

QUESTIONNAIRE

ABOUT

DIAGNOSIS

This questionnaire contains a number of specific Dr questions which Duffey, Neurological Consultant phil Hospital, at York General and to in Newcastle Victoria Infirmary used work at the Royal who would to like ask treatments you about your past consultations, and Dr Duffey has worked diagnoses. the team closely with research 1993 on all the programmes. interested He is to learn how since diagnosis of dystonia your came to be made and in particular who to see when your first you were advised condition arose. Questions

Tick

is

today's

00)

What

ol)

To which referred,

Date

of the before

Orthopaedic

Surgeon

02)

specify)

an Osteopath

.......

-o

number

were ?

is

181

you

...

.....................

treatments ? or tried -.

for

your

0 ........................

............. ......

Physiotherapy

o ................... 0 .....................

................................

Psychotherapy

03)

.........

................................

Hypnosis

(please

code

Office

.........................

Acupuncture

Other

Your

.........

What physical or alternative have been advised condition Use of

here

comment

.................................

Psychiatrist (please

:

or

following medical specialists your dystonia was diagnosed

Rheumatologist

other

answer

................................ specify)

How long before the begin your symptoms

................... correct ?

diagnosis

o ............ was made did

.............................. Thank you for the information I hope that your help. you have provided may make the diagnosis of future patients With dystonia difficult to Dystonia easier. can be very diagnose is not my intention to imply and it criticism of those involved in your is a Hindsight particular case. wonderful ! tool Dr P. Duffey, MB. BS., MRCP. Page 1

FIRST

QUESTIONNAIRE

DNP EVALUATION

-

Office 00) Today's

is

Date

Your code number is Write your answers

.............

below

01)

02) 03) 04)

how many times Can you estimate you been injected since you first started How many different injections those How much time to spend with

have people since you first

has How many times you the injections

to answer Rating Scale 1= Very good 2= Good 05)

06)

07)

to tell you like the service about

..............

?

......... giving ?

their

At ?

How would their you rate towards personal attituýle ? you and your condition

.

.........

........

Questions Numbers 5 and 6 3= Average, 4= Poor, 5

How would you rate their knowledge of you and your particular condition

Would think

?

able

in technique or been painful

their hurt

.........

given you started

they generally (in minutes)

are you

have ?

their

At

their

Poor

........

worst

........

best

their

At At

best

Very

........

worst

........

me (in your own words) you get from Hunters

what Moor

you

........................................

...................................................... ...................................................... 08)

What

type

of

muscle

spasm(s)

do you

have

?

...................................................... 09)

Do you If,

10)

Is

know

YES, there

what

is

the

is

it

?

what

anything

which

cause

of

.....

this ......

type

of

spasm

0 ...............

makes

these

spasms

worse

?

makes

these

spasms

better

?

...................................................... 11)

Is

there

................

anything

which

o .....................................

?

191

SECOND QUESTIONNAIRE

DNP EVALUATION

-

Office is

00)

Today's

01)

How many times since you last

02)

03)

04)

Date

............. you have answered

How many different injections those time with

How much to spend How you

06)

07)

injected

been this

they (in

are you

many times injections the

has

generally minutes)

like to service

tell you

you

able ?

.........

their

At

their

At

me (in currently

........ Very

best

........

best

........

worst

........ how you improved

your own words) be get could .

................................

Poor

........

worst

their

At

giving ?

5 and 6 Poor, 5

their

At ?

.........

.........

Questions Numbers 3= Average, 4=

How would their you rate towards personal attitude ? you and your condition you the

given

in technique or been painful

their hurt

their How would you rate knowledge of you and your particular condition

Would think

?

questionnaire

have people then ? since

to answer Rating Scale Very 1= 2= Good good 05)

is Your code number Write your answers below

......................

...................................................... ...................................................... 08)

Do you

have

primary

or

secondary

dystonia

primary

dystonia

?

...................................................... 09)

What

is

thought

to

cause

?

...................................................... 10)

What

are

one

of

the

causes

of

secondary

dystonia

?

...................................................... 11)

What

is

the

main

difference

......................................................

between

the

two

types

?

192

THIRD

QUESTIONNAIRE

DNP EVALUATION

-

Office

is

00)

Today's

01)

How many times since you last

02)

Date

Your code number is Write your answers below

............. you have answered

How many different injections those

injected questionnaire

been this

have people ? then since

given

193

+--+

?

II

.........

+--+ +--+

you

+--+

03)

How much time to spend with

they generally (in minutes)

are you

?

+--+

able

+---+

04)

has How many times you the injections

to answer Scale Rating Very 2= 1= Good good

in technique or been painful

their hurt

Questions Numbers Average, 3= 4=

+--+

giving ?

+--+

5 and 6: Poor, 5=

Very

Poor +--+

05)

their How would you rate knowledge of you and your particular condition

best

their

At

II II II

+--+

?

At

their

worst

+--ý

06)

their How would you rate towards personal attitude ? you and your condition

best

their

At

+--+

At

their

worst +---+

07)

to tell you like the service about

Would think

me (in your own words) you get from Hunters

what you Moor :

II

+--+

+--+

+--+

08)

What

is

meant

by

idiopathic

?

dystonia

+--+

+--+

09)

What

is

the

?

basal-ganglia

+--+

+--+

10)

Do you think to compared

your treatment the treatment

has improved you got a year

ago

? YES

How do you the service Circle

one

rate you

answer

the service compared received a year ago BETTER

/

/

NO

to ?

ABOUT

THE

SAME

WORSE

FOURTH

QUESTIONNAIRE

DNP EVALUATION

-

Office 00)

01)

Today's

is

Date

Please tell me in the whole project

.............

Write

your own regarding

Your your

words, what you think the Nurse Practitioner

..................................

0 .........

.o........................

03)

..........

If you prefer

independent have the to be injected at

If

prefer

you

to

be

....................

If

you

to

prefer

.... .......

injected

at

would you the clinic

home,

?

why

?

..........

be

injected

o ............ at

the

clinic,

why

?

.... . ..

......................................

.................. Is there anything

choice, home or in

. . ....................

..............

05)

0 .................

............................

................... 04)

about :

o ...................

.........................................

02)

194

is code number below: answers

............................ anything you would

else like

you us

o .......

like to would to know about

talk this

about project

............................................. . oo-

0 .......

.........

............................. o .................. ............................. ........................

?

o

.................

...............

or

.........

0 .................. o ..................

0 .........

0 .................... o ........................ 0 ......

0 .................

0.. 0 .....

Dystonia -A comprehensiveand longitudinal study in the North East of England

APPENDIX

C

SPSSCODING FILE The PhD SPSSfile entitled 'phddata.sav' contains6 yearsdata from 6th May 1993to 5th May 1999,which has also beenused on other associatedresearchprojects :ESD = CUA = EPD = DNP = PSR = IEFD = SER = EST =

Epidemiological Survey of Dystonia - all data availableat the time Cost Utility Analysis - No 00 1 to 23 1 inclusive (seenote re patient numbering) Epidemiology of Primary Dystonia -'if aet = I'- all data at the time Dystonia Nurse Practitioner - all data on 126 selectedcases Psycho-SocialResearch- all data to 550 + 564,594 and 677 Dystonia affecting working musicians- only 3 selectedcases Socio-Econon& Research- all data availableat the time Epidemiology of SpasmodicTorticollis - 001 - 766 inclusive

Patient Numbering and Status used in 'phddata. sav'. The patient's ID number is automatically determined by the row number and the column shown as ESD is used for ranking purposes only. NB : Six CUA patients have been removed and replaced by ESD patients between 04.12.95 and 04.03.96. They and 210, who were all non-dystonic CUA controls. were No's 148,152,159,170,175

00 1 to 199 inclusive: were active CUA patientscodedA, B or D 200 : is a new ESD patient codedM 201 to 231 inclusive: were external control CUA patients,re-codedto ESD 232 to 300 inclusive: are new ESD patients coded A, B or M 301 to 347 inclusive: were (1994) 500 Seriespatientsre-numberedto 300 348 to 937 inclusive: are new ESD patients numberedand coded accordingly Syntax Files Syntax file copieshavebeen enclosedseparatelybut most detailed formulae have been included in the coding file. The 7 syntax files were run in the following order: phd-geog.sps phd-all.sps phd-dec.sps phd-sf36.sps phd-qual.sps phd-psr.sps phd-env.sps

Recoding 'post codes'into 'county' and 'district'. 21 computationsre Age, DOB, Dates, Differences,etc 12 computationsre defining decimalplacesin the above. 29 computationsre EuroQol and SF36as per handbook. 3 computationsre qualitative open-questions. 17 computationsre PSR, as per Dr Jahanshahi'sinstructions. 93 comps 'if env = 1, envaa= 0,' etc., & 'if alg = 2, alga = 2,' etc.

SPSS Coding Frame The SPSS codes (up to 9 275) were used in the Cost Utility Analysis (CUA) but have since been amended for the ESD, EST, IFD, EPD, SER, PSR and DNP, as required. All variables are entered as Numeric 4.0; missing values None; alignment Right and column width 4, unless otherwise shown. The value label positions, ie h!, are numbered (subjects) file". file list The 885 "the the contains rows as per working of variables on data has 745,170 (variables) 842 total therefore a of sets. potential x columns and

52

Dystonia -A comprehensiveand longitudinal study in the North East of England

#

Name

Variable Label

Additional Info

001 esd

ESD Patient ID number

002 geogI

County of Residenceof ESD subject calc. from post codes- seesyntaxfile: 'phd-geog.sps' 10 = Co. Durham 20 = Cleveland(Teesside) 30 = Tyne & Wear 40 = Northumberland 50 = N. Cumbria 60 = S. Cumbria 70 = N. Yorks 80 = Rest of Yorkshýire 90 = Other Areas in UK

003 geog2 District (AdniHstrative) -see syntaxfile: 'phd-geog.sps' 10 = Co. Durham II= ChesterLe Street

consistsof

12= Darlington 13= Derwentside 14= Durham 15= Easington 16= Sedgefield 17= Teesdale 18= WearValley 20 = Cleveland(Teesside)consistsof 21 = Hartlepool 22 = Middlesbrough 23 = Redcar & Cleveland 24 = Stockton on Tees 30 = 31 = 32 = 33 = 34 = 35 =

Tyne & Wear Gateshead Newcastle North Tyneside South Tyneside Sunderland

consists of

40 = Northumberland consistsof 41 = Alnwick 42 = Berwick upon Tweed 43 = Blyth Valley 44 = CastleMorpeth 45 = Tynedale 46 = Wansbeck 53

Dystonia -A comprehensiveand longitudinal study in the North East of England

50 = N. Cumbria 51 = Allerdale 52 = Carlisle 53 = Eden

consistsof :-

60 = S. Cumbria 61 = Barrow 62 = Copeland 63 =S Lakeland 64 = Lanchester

consistsof :-

70 = N. Yorks 80 = Rest of Yorkshire 90 = Other Areas in LJK 004 hospI

Main Hospital (alpha) - Patient registeredat

String 4

A= NRI North Riding Infirmary, Middlesbrough B= HMH Hunters Moor Hospital, Newcastle C= CUA Patient in CUA living outsidethe region, unknown hospital D= More Patient registeredat more than I Hospital in the region E=ESD Patient in ESD living outsidethe region, unknown hospital F=SSG South ShieldsGeneralHospital G= MGH Middlesbrough GeneralHospital H= HGH Hartlepool GeneralHospital J= FHN FreemanHospital, Newcastle K= BAH Bishop Auckland Hospital L= LGH Leeds GeneralHospital M DN4H Darlington Memorial Hospital N NGH Newcastle GeneralHospital R RVI Royal Victoria Infirmary, Newcastle S= SGH SunderlandGeneral(& Royal) Hospital T= SEI SunderlandEye Infirmary X UNK Patient in ESD living insidethe region, unknown hospital Y DRY Drybum Hospital, Durham 005 hosp2 Second Hospital (ic. patient's notes also availablefrom) Variable codesas above 006

hosp3

A+B = A+M = B+N = G+N = M+G= T+S =

7 2 7 3 I I

Combined Hospitals (for 135 Upatients as of 31.12.98) NB : D' patients were only shown in the CUA, but the details are

A+G A+R B+R K+A M+N T+Y

= II = I = 39 = 3 = I = 2

A+H B+A B+S K+B N+B S+N

= 2 = 16 = 5 = I = 3 = I

A+K = I B+J = I B+Y = 8 M+A = 3 R+B = 9 Total = 135

A+L B+K G+A M+B T+B

= = = = =

2 I 2 I I

54

Dystonia -A comprehensiveand longitudinal study in the North East of England

007 hosp4 Main Hospital (num) As per # 004 but numeric for filter purposes,etc. I =NRI 2= HMH 3= CUA 4= More 5 =ESD 6=SSG 7= MGH 8= HGH 9= BAH I O=LGH I 1= DMH 12= NGH 13= RVI 14= SGH 15= SEI 16= UNK 17= DRY 18= FHN

North Riding Infirmary, Middlesbrough Hunters Moor Hospital, Newcastle Patient in CUA living outsidethe region, unknown hospital Patient registeredat more than I Hospital in the region Patient in ESD living outsidethe region, unknown hospital South ShieldsGeneralHospital Middlesbrough GeneralHospital Hartlepool GeneralHospital Bishop Auckland Hospital Leeds GeneralHospital Darlington Memorial Hospital Newcastle GeneralHospital Royal Victoria Infirmary, Newcastle SunderlandGeneral(and Royal) Hospital SunderlandEye Infirmary Patient in ESD living insidethe region, unknown hospital Drybum Hospital, Durham FreemanHospital, Newcastle

008 CMI

Clinical Registration Number of the patient at their Ist hospital (if known)

String 8

010 crn2

Clinical Registration Number of the patient at their 2nd hospital (i f known)

String 8

0 12 surv

Survey Participation

Numeric 4.0

1= Full participation 2= Part participation 3= Non participation 4= ESD data only 5= After closing date 6= Died before (non) 7= Died during (part) 8 =Died since (full) 9 =Died since (non)

- completedeverythingpossible but completed most, not all q'res declined, data ESD only collected to not asked participate date ESD 31.12.1998 the closing was interview data ESD only no - someq'res completed if most q'res completed, not all. interview data ESD no only -

55

Dystonia -A comprehensiveand longitudinal study in the North East of England

EuroQol Questionnaire: Q'res No. I or 10 0 13 date10 Date of Q're 1 or date of first contact or date enteredon SPSS

dd.mm.yy 8

014 agelO Age (of patient) at datelO 0

Numeric 4.1

Compute agelO = YRMODA (xdate.year(datelO),xdate.month(datelO), date.mday(date10)) /3 65.25 YRMODA (date.year(dob),xdate.month(dob), xdate.mday(dob))/365.25. Execute. ComputeagelO = (agelO + 0.5) (MOD((age 10 + 0.5),l)). Execute. NB : This formula computesand shows agelO with no decimalplaces.

015 statl

Statusof Q're I= 2= 3= 4= 5= 6= 7= 8= 9=

016 mobl.

usedin ESD only

have no problems(in walking) I have someproblems(in walking) I am confined to bed (wheelchair)

relatesto Q. 02: Self-care I=I

018 actl

usedin CUA only

relatesto Q. 01: Mobility I=I

017 self].

Injected today date NEd-InjectionCycle Irj. stoppedduring (study) Never (been)injected Irj. stoppedbefore (study) In remission Q're not returned Unknown status Q're not issued

Numeric4.0

have no problems(re self-care) I have someproblems(washing) I am unableto wash (or dress)

relatesto Q. 03: Usual activity I=I have no problems 2=I have someproblems 3= Am unableto perform (usual activity)

019 disel.

relatesto Q. 04: Pain / discomfort I=I

have no pain (or discomfort) I have moderatepain (or discomfort) I have extreme pain (or discomfort) 56

Dystonja -A comprehensiveand longitudinal stucýyin the North East of England

020 anxI

relatesto Q. 05: Anxiety / depression I=I am not anxious(or depressed) 2=I am moderatelyanxious(or depressed) 3=I am extremelyanxious (or depressed)

021 ostatel EuroQol Own Status I (EuroQol calculation) seesyntax file : 'phd-sf36.spsfor formula 11111=All I -noproblems 22222 = All 2- someproblems 33333 = All 3- severeproblems 022 ml 023 sl 024 al 025 dI 026 X1

Mobility CaIcs I Self-care Cales 1 Usual Act Calcs 1 Pain / Disc. Caics I Anx. / Dep. Calcs I

027

EuroQoI Score I (HRQoL = Health-related Quality of Life)

euro I

100 46.81 -17.94 028

heall

(Missing (Missing (Missing (Missing (Missing

Value= Value = Value = Value = Value =

9.000) 9.000) 9.000) 9.000) 9.000)

Best HRQoL score =IIIII = 22222 Average HRQol score = 33333 Worst HRQoL score

relates to Q. 06: Health State Visual Analogue Scale (VAS) from 0 to 100

SF 36 Questionnaire: Q'res No. I or 10 029 genI

relatesto Q. 07: General Health

Numeric 4.0

NB : Where variablelabels are shown with (numbers),it indicatesthat the variable has beenrecodedprior to SF36 calculationsin accordancewith the manual "How to score the SF-36Health Survey." 5= 4= 3= 2= 1=

Excellent (1) Very Good (2) Good (3) Fair (4) Poor (5)

= = = =

100 84 61 25 0

Recodeda secondtime as per instructions on Page 17 of the abovemanual. ( Final result 0- 100 )

030 compl. relatesto Q. 08: Compared to I yr. I= 2= 3= 4= 5=

Much better Better About same Worse Much worse 57

Dystonia -A comprehensiveand longitudinal study in the North East of England

031

vigl

relates to Q. 09a: Vigorous Activity

I= Yes lin-ýiteda lot 2= Yes linýiteda little 3= Not linýitedat all 032 033 034 035 036 037 038 039 040

modl liftl SeVI onel bendI walkI walb]. walel. bathl.

relatesto Q. 09b: Moderate Activity relatesto Q. 09c: Lifting / carrying relatesto Q. 09d: Climbing several relatesto Q. 09e: Climbing one relatesto Q. 09f Bending, kneeling relatesto Q. 09g: Walking more mile relatesto Q. 09h: Walking half mile relatesto Q. 09i: Walking 100 yards relatesto Q. 09j: Bathing / dressing

Template= Vigorous

041 pfl

Physical Functioning 1 (syntax: 'phd-sf36.sps') 0= Low PhysicalFunctioning 100 = Fligh PhysicalFunctioning

042 cut].

relatesto Q. 10a: Cut down I =Yes 2= No

Template= Yes / No

043 accl 044 liml. 045 hadl

relatesto Q. I Ob:Accomplished less relatesto Q. 10c: Limited in kind relatesto Q. 10d: Had difficulty

Template= Yes / No

046 rpl.

Role - Physical 1 (syntax: 'phd-sf36.sps') 0= Worse Role - Physical 100=BetterRole -Physical

047 timel 048 lessl 049 work].

relatesto Q. I la: Amount of time relatesto Q. IIb: Accomplished less relatesto Q. I le: Didn't do work

050 rel

Role - Emotional 1 (syntax: 'phd-sf36.sps) 0= Worse Emotional Functioning 100= Better Emotional Functioning

051 extI

relatesto Q. 12: To what extent has 5= 4= 3= 2= 1=

Template= Yes / No

Template= Extent

Not at all (1) Slightly (2) Moderately (3) Quite a bit (4) Extremely (5)

58

Dystonia -A comprehensiveand longitudinal study in the North East of England

052 howl

relatesto Q. 13: How much bodily 6= 5= 4= 3= 2= 1=

None (1) Very n-ffld(2) Mild (3) Moderate (4) Severe(5) Very severe(6)

053 painI - relatesto Q. 14: How much did pain Recodedas extent 4051 054 bpl

Template= How

Template= Extent

Bodily Pain I (syntax: 'phd-sf36.sps!) 0 =Most Bodily Pain 100= Least Bodily Pain

055

lifel.

relates to Q. 15a: Full of life

Template = Life

6= All of the time (1) 5= Most of the time (2) 4= Good bit of the time (3) 3= Someof the time (4) 2=A bit of the time (5) 1= None of the time (6) 056 057 058 059 060 061 062

nervI downl calml. IOU low I woml happI

063 mhl

relatesto Q. l5b: Very nervous relatesto Q. 15c: Have you felt down relatesto Q. 15d: Have you felt calm relatesto Q. l5e: Did you have a lot relatesto Q. 15f. Have you felt low relatesto Q. 15g: Did you feel worn relatesto Q. l5h: Are you happy

Not recoded Not recoded Recoded Recoded Not recoded Not recoded Recoded

Mental Health I (syntax : 'phd-sf36.sps) 0= Worse Mental Health 100= Better Mental Health

064 tirl

relatesto Q. 15i: Did you feel tired

065 vtI

Vitality 1 (syntax: 'phd-sf36.sps')

Not recoded

0= Least Vitality 100 =Most Vitality

066 socl.

relatesto Q. l5j: Social activities

Not recoded

59

Dystonia -A comprchensiveand longitudinal study in the North East of England

067 sfI

Social Functioning I (syntax: 'phd-sf36.sps) 0= Worse Social Functioning 100=Better Social Functioning

068 illl

relatesto Q. l6a: I get ill more I= 2= 3= 4= 5=

069 anyl

Definitely true Mostly true Not sure Mostly false Definitely false

relatesto Q. l6b: As anybody 5= 4= 3= 2= 1=

Template= III

Recodedas below

Definitely true (1) Mostly true (2) Not suFe(3) Mostly false (4) Definitely false (5)

070 worl 071 excl

relatesto Q. 16c: Health to get worse relatesto Q. 16d: Health is excellent

072 ghl

General Health I (syntax : 'phd-sf36.sps) 0= Worst GeneralHealth 100= Best GeneralHealth

073 typel

Type of Bot Tox used - Info from Clinical Analysis Form (CAF) I= 2= 3= 7= 8= 9=

074

dose I

Not recoded Recoded

Speywood- Dysport Allergan - Botox Both types used No longer injected Unknown type Never beeninjected

Dosage of Bot Tox -from CAF

075 sitel

Number of sites - on nearestdate to Qre from CAF

076 partl

relatesto Q. 17: With partner

Template= Partner

I= Extremely positive 2= Positive 3= 4= 5= 6= 7=

Slightly positive Neutral (missing) Slightly negative Negative Extremely negative

NfissingVariable =4

60

Dystonia -A comprehensiveand longitudinal study in the North East of England

077 078 079 080 081 082 083

peopI burdI embI appI conI copI supI

084 quall

relatesto Q. 18: With people relatesto Q. 19: Felt a burden relatesto Q. 20: Embarrassed relatesto Q. 21: Appearance relatesto Q. 22: Control relatesto Q. 23: Coping with life relatesto Q. 24: Support

Template= Partner

Qualitative Data 1 8= Maximum +ve 20 = Average +ve 0 32 = Neutral 44 = Average -ve 56 = Maximum -ve

Computequall = partl + peopl + burdl + embl + appl + conl + copl + supl. Execute.

EuroQol Questionnaire: Q'res No. 2 or 11 085 datel I

Date of Q're 2 or date of last known injection.

dd.mm.yy

086 time

time from Q're 10 to Q're 11

Numeric 4.1

Compute time = YRMODA (xdate.year(datel 1),xdate.month(datel 1), xdate.mday(datel 1)) / 365.25 - YRMODA (xdate.year(datel 1),xdate. month(datell), xdate.mday(datell))/365.25. Execute. Computetime = (((time * 10)+0.5)-(MOD(((time * 10)+0.5), 1)))/10. Execute. 087 recode2 time between Q're 10 and 11. I= Over 3.50 years 2=3.00 - 3.49 yrs 3=2.50 - 2.99 yrs 4=2.00 - 2.49 yrs 5=1.50 - 1.99 yrs 6=1.00 - 1.49 yrs 7=0.50 - 0.99 yrs 8=0.00 - 0.49 yrs 9= Negative years All variablesare then repeatedfor the rest of Q're No. II (ie EuroQol 2 and SF36 2) and duplicatesall the variablesas for Q're No. 10 from # 088 until # 157. 158 qua13 Qualitative difference over time Compute qual3 = qualI- quall Execute. A positive scoremeansthe subjectis feeling better and a negativescore meansthey are feeling worse over the period of time between 10 and I I. defined by 4087. 61

Dystonia -A comprehensiveand longitudinal study in the North East of England

Demographic Questionnaire: Q're No. 20 159 date20 Date of Qre No 20

dd.mm.yy 8

160 age20 Age of patient at date20

Numeric 4.1

Compute age20= YRMODA (xdate.year(date20),xdate.month(date2O), xdate.mday(date20))/ 365.25 - YRMODA (xdate.year(dob),xdate.month (dob),xdate.mday(dob))/ 365.25. Execute. Compute age20 = (((age20 * 10) + 0.5) (MOD(((age2O * 10) 0.5), 1)))/10. + Execute.

161 gend

relatesto Q. 01: Gender

Numeric 4.0

1= Male 2= Female 162 dob

relatesto Q. 02: Date of Birth

163 agel

Age at 01.01.99 (ie end of epidemiology)

dd.mm.yy 8

Compute agel = YRMODA (98,13,00) / 365.25 YRMODA (xdate.year(dob) xdate.month(dob),xdate.mday(dob)) / 365.25. Execute. Compute ageI= (ageI+0.5) - (MOD((age I+0.5), 1)). Execute. 164 post

String 4 relatesto Q. 03: Home Post Code (2 alpha+4 numeric, eg DL2 1= DL02 1) Align left NB : Post Codeswithin EPD and Cumbria to SectorLevel (seelist at end) 165 mar

relatesto Q. 04: Marital Status I= 2= 3= 4= 5= 6=

166 167 168 169

malel. fernI male2 fern2

170 eld 171 yng

Numeric 4.0

Single Married Cohabiting Separated Divorced Widowed

relatesto Q. 05: No of male children relatesto Q. 05: No of female children relatesto Q. 05: Male kids in house relatesto Q. 05: Female kids in house relatesto Q. 05: Eldest's birth year 00 = 1900,then includesevery year to 96 = 1996. relatesto Q. 05: Youngest's birth year 00 = 1900,then includesevery year to 96 = 1996. 62

Dystonia -A comprehensiveand longitudinal study in the North East of England

includesDemographic Questionnaire: Q're No 25 Q're updating demographicinformation dd.mm.yy 8

172 date25 Date of Q're 25 173 age25

Numeric 4.1

Age at date25

Compute age25= YRMODA (xdate.year(date25),xdate.month(date25), (xdate. / 365.25 YRMODA year(dob),xdate.month mday(date25)) xdate. (dob),xdate.mday(dob))/ 365.25. Execute. Compute agge25= (((age25 * 10) + 0.5) - (MOD(((age25 Execute. 174 difF5

* 10) + 0.5), 1)))/10.

Numeric 2.1

Time from 20 to 25 (in years)

Compute diff5 = age25- age 20. Execute. Compute diff:5)= (((diff5 * 10) + 0.5) - (MOD(((diff5 * 10) + 0.5), 1)))/10. Execute. 175 empl

Numeric 4.0

Status 20 Q. Employment 06: to at relates I= 2= 3= 4= 5= 6= 7= 8= 9=

Full time employment Part time employment Unemployed Self Employed Unwaged Retired OAP Retired ill health On long term sick Deceased

Temp = Employ

176 emp2

Status 25 Q. Employment 06: to at relates

177 segI

SEG Template SEG Patient 20 Q. 07: = to at relates I =A: Professional Managerial 2=B: 3= CI: White Collar (Skilled Non-Manual) C2: Blue Collar (Skilled Manual) 4ý Semi-skilled (Unskilled) 5=D: (Unwaged, Studentor Unemployed) Retired 6=E: Deceased 9=

178 seg2 179 seg3 180 seg4

25 SEG Patient Q. 07: to at relates relatesto Q. 07: SEG - Patient (highest ever) (current) SEG Head House Q. 08: to of relates taken to be amale'Head of House, if widow =9 House (highest Head SEG Q. 08: to ever) of relates taken to be a'male'Head of House, if widow, not =9

181 seg5

63

Dystonia -A comprehensiveand longitudinal study in the North East of England

The above SEG codesare defined accordingto Reid (1989) as :A: Upper NEddleClass. Defined as successfulbusinesspersons(eg, self-employed/ manager / executive of large enterprise), higher professionals(eg, bishop, surgeon, medical specialist, barrister, accountant), senior civil servants (above Principal) and local governmentofficers ( eg, chief, treasurer,town clerk). Known as Professional B: Middle Class. Defined as senior, but not the very top, people in the same classificationas A. Known as Managerial CI: Lower NEddle Class. Defined as small tradespeople, non-manual, routine administrative, supervisory and clerical (sometimes referred to as 'white-collar' workers). C2: Skilled Working Class( often referred to as 'blue-collar') D:

Senii-skilledand unski-Iledworking class

E: Those at the lowest levels of subsistence,including the retired, those on social have I because and also of sickness or unemployment, casual workers. security included studentsin this category. The national percentagesof informants falling into theseclassesare :A= B= C, C2 D= E=

3% 14% = 22% = 28% 18% 15%.

The reasonthat this classificationhas been used is it is the only one is common usage that has the non-employedcategofy of retired, on disability benefit or income support.

182 inel

relatesto Q. 09: Patient's Income at 20

183 inc2

income Q. Patient's 09: to at 25 relates

Comma6.0

NB : If there is no changein income shown on Qre 25, then the following formula is used to calculatefor inflation -If diff5 = 3.2 to 2.7 yearsthen inc Ix1.10 = inc2 with 10% added-38.4% of study. 28.3% 7% 2.6 to 1.6 x 1.07 1.5 to 0.6 0.5 to 0.0

x 1.03 x 1.00

3% 0%

5.8% 27.5% -------------100.0%

64

Dystonia -A comprehensiveand longitudinal study in the North East of England

184 benel. relatesto Q. 10: Benefits - 1st Numeric 4.0 1 = SicknessBenefit 2 = Invalidity Benefit (Incapacity) 3 = AttendanceAllowance 4 = Mobility Allowance 5 = OccupationalPension 6 = Retirement/ Widows (Pension) 7 = Income Support 8 = DisabledWorking (Allowance) 9 = Loss of Earnings (Benefit) include in UnemploymentBenefit 10 = Invalid Care (ICA) II = DisabledLiving (DLA) 12 = SevereDisability (Allowance) 13 = MOD Disability (War Pension) 14 = CancerVictim (Allowance) 15 = Child Benefit (Family Allowance) 16 = Family Credit 17 = Industrial Injury (Benefit) 18 = Wagesin prison 19 = Rent Allowance 185 bene2 relates to Q. 10: Benerits - 2nd Template = Benefits 186 bene3 relates to Q. 10: Benerits - 3rd $benetot Total of Benerits - variables benel, bene2 and bene3 Multiple Response Set - Range I to 19 187 188 189 190 191 192

hourl hour2 dayl day2 nýlel i-nile2

relatesto Q. 11: Patient - hours off to attend hosp. relatesto Q. 12: Helper - hours off to accompany.pat. relatesto Q. 13: Patient - days off in last 6 weeks. relatesto Q. 14: Partner - days off in last 6 weeks. relatesto Q. 15: No of miles -I way (old hospital) relatesto Q. 15: No of miles to new hospital 0= Home visit by DNP (Dystonia Nurse Practitioner) 193 minsl relatesto Q. 16: No of minutes -1 way (old hospital) 194 nýns2 relatesto Q. 16: No of minutes to new hospital 0= Home visit by DNP (Dystonia Nurse Practitioner) 195 travI

relatesto Q. 17: Method of travel I = Own Car 2 = Family Car 3 = Friend's Car 4 = Public (Transport) mainly Bus 5 = Public (Transport) mainly Train 6 = Ambulance/ Hosp Car (Prison Van) 7 = Taxi 8 = Bicycle 9 = Walked or pushed(in wheelchair) 10 = Home visits - DNP II = Aeroplane

Template = Travel

65

Dystonia -A comprehensiveand longitudinal study in the North East of England

196 trav2

relatesto Q're 25 : Method of travel

TemPlate= Travel

197 drl

(Name oo Dr who diagnosed

Template= Doctor

0= Not yet diagnosed I= Hawthorne - NRI 2= Bames - FIMH 3= Duffey - ESD et al 4= Allchin - SEI 5= Bums - RVI / DRY 6= Tilley - DMII / MGH 7= Newman - MGH 8= Saunders- MGH et al 9 =Unknown at HMH 10 = Unknown at NRI II= Prof Walton 12 = DNP study patient 13 = Bates - RVI / Cumbria 14 = Cartlidge - NGH 15 = G-Medwin - NGH 16 = Hudgson - NGH 17 = Jordan- RVI / BMH 18 = Prof Marsden 19 = Foster - RVI 20 = Unknown Dr 21 = Cleland- SGH 22 = Shakir - MGH / HGH 23 = Shaw - RVI 24 = Jones- SGH 25 = Walls - NGH 26 =Prof Nattrass/S eggar 27 = Unknown at MGH / HGH 28 = Unknown at NGH 29 = Unknown at RVI 30 = Field - HMH 31 = Bincloff - MGH 198 dr2

(Name oD 2nd Dr to diagnose

199 aetl

relatesto Q. 18: Aetiology of Dystonia (old) I= Primary (Idiopathic) 2= 2ndary (Symptomatic) 3= Not dystonia(control) 4= HFS

Template= Doctor

The aetl classificationwas in existence at the start of the study in 1993 and used throughout all the fieldwork. It was amendedin October 1996 at the 3rd International Dystonia Symposiumin Miami. The aet2 classificationwas added to ESD in January 1998 and all the 838 subjectswere re-classifiedaccordingto the new codes. 66

Dystonia -A comprehensiveand longitudinal study in the North East of England

200 aet2

relatesto Q. 18: Aetiology of Dystonia (new) 10 = Prima1y Dystonia ie II= Oppenheim's 12 = Fam. Torticollis 13 = Fam. Cerv-Cran. 14 = Other Fam. (familial) 15 = Sporadic 20 = 12ystonia-Plus ie 21 = D. R. D. 22 = Myoclonic 23 = D. R. D. (familial) 24 = Myoclonic (familial) 30 = Seconda!y Dystonia - caused by 31 = Levo-Induced 32 = Tardive Dystonia (including neuroleptic induced) 33 = C.P. 34 = M. S. 35 = Infectious 36 = B. E. T. 37 = Cerebrovascular 38 = Metabolic ? 39 = Head Trauma (Head Injury) 40 = Heredodegenerative Diseases ie 41 = M. S.A. 42 = Shy Drager's (Syndrome) 43 = Leigh's (Disease) 44 = P.D. 50 = Other Dystonias (not shown above) ie 51 = Tardive Dyskinesia 52 = Paroxysmal Choreo-athetosis 53 = Paroxysmal Dystonia 54 = Paroxysmal (Familial) 60 =Not Dystonia, ie controls with 61 = HSP 62 = CP 63 = Myoclonus 64 = BET 65 = Peripheral Neuropathy 66 = Ulnar Nerve Palsy 67 = Chorea 69 = ME 69=MS 70 = PD 71 = Arthrogryposis 72 = SSS (Spasticity secondary to Stroke) 80 = HFS 90 = Undefinable (as this time) 91 = Psychoizenic-(in nature)

67

Dystonia -A comprehensiveand longitudinal study in the North East of England

201 prim

relatesto Q. 18: Primary Dystonia (old aetiology) I= 2= 3= 4=

202 sec

Idiopathic (ITD) Dopa Responsive(DRD) Paroxysmal Myoclonic

relatesto Q. 18: Secondary Cause (old actiology) I= CerebrovascularAcc (CVA) 2= Amold Chiari (Malformation) 3= Aneurysm 4= CerebralPalsy 5= Drug Induced 6 =MS 7= MSA 8= Parkinson!s (Disease) 9= Psychogenic(in nature) 10 = Meningitis / Encephalomyelitis II= Shy Drager's (Syndrome) 12 = Stroke (Spasticity) 13 = Tardive Dvskinesia(Dystonia) 14 = Leigh's Disease 15 = Metabolic ?? 16 = BET 17 = HFS (unknown cause) 18 = Undiagnosed(or undefinableat this time) 19 = HFS (2nd operation) 20 = SAH (SubarachnoidHaemorrhage) 21 = Stroke (dystonic) 22 = 23 = 24 = 25 =

IUS (2nd CVA) HFS (Aneurysm) HFS, (2nd D. I. ) HFS (2nd MS)

203 notdys Relatesto conditions wMch are not dystonia 61 = HSP 62 = CP 63 = Myoclonus 64 = BET 65 = PeripheralNeuropathy 66 = Ulnar Nerve Palsy 67 = Chorea 68 = MIE 69 = MS 70 = PD 71 = Arthrogryposis 72 = SSS(Spasticity secondaryto Stroke) 68

Dystonia -A comprehensiveand longitudinal study in the North East of England

204

distl

relates to Q. 18: Distribution I= 2= 3= 4= 5= 6= 7= 8=

205

focl

206 foc2 207 foc3 208 foc4

Focal dystonia Segmental dystonia Multi-focal dystonia Generalised dystonia Hemi-dystonia Undefined spasms Other neuro disorder Undiagnosed spasms

Temp = Distribution FD (abbreviation) Seg MF GD HD

relates to Q. 18: Focal dystonia I

Template = Focal

I= 2= 3= 4= 5= 6= 7= 8= 9= 10 = II= 12 = 13 = 14 = 15 =

BL (abbreviation) OMD SD ST RT AT HC or WC LD Fac DD FD - leg/ ft FD - arm/hd HFS Orofac Trnk

Blepharospasm Oromandibular Spasmodic Dysphonia (Laryngeal Dystonia) Spasmodic Torticollis Retrocollis Antecollis Hand (Writers) Cramp Lingual Dystonia (Tongue not Laryngeal) Facial Dystonia Dystonic Dysphagia, Leg (Foot) Dystonia Arm (Hand) Dystonia Hemi-facial Spasm Orofacial dystonia Trunk

relatesto Q. 18: Focal dystonia 2 relatesto Q. 18: Focal dystonia 3 relatesto Q. 18: Focal dystonia 4

Template= Focal

$fOctot Total of Focal Dystonias - variablesfocl, foc2, f6c3 and foc4 Multiple ResponseSet - Range I to 15 209 sgmt

relatesto Q. 18: Segmental dystonia I= 2= 3= 4= 5= 6= 7= 8=

Cranial Axial Brachial Crural Craniocervical Meige's Syndrome (Cranial) Breughel'sSyndrome(Cranial) Cervical (both focal dystoniasin the neck area)

69

Dystonia -A comprehensiveand longitudinal study in the North East of England

2 10 side

relatesto Q. 18: Side affected I =Left 2= Right 3= Both sides 4= Forward (neck) 5= Backward (neck) 6= Tremor (shaking) 7= Diagonally opp sides 8= Undefined side

211 onsetI

relatesto Q. 19: Year of onset from the interview 00 = 1900, then includesevery year to 98 = 1998

212 age2 Age at onset Compute age2 = onset I+ 1900 xdate. year(dob). Execute. -

213 diag

relatesto Q. 20: Year of diagnosis from the interview 00 = 1900, then includes every year to 98 = 1998

214 age3 Age at diagnosis Compute age3= diag + 1900 - xdate.year(dob). Execute. 215 diffl Difference onset-diag, ie Years to correct diagnosis Compute diffl = age3- agel Execute. NB : The difference in years should be correlated with the original diagnosis. Some patients did not seekearly advice as their dystoniawas not severeat first. Seevariable label 9286 'del' - ie the reasonfor any delay in diagnosis. 'diffl' should not be used in isolation but must be seenin context. 216 trig'

relatesto Q. 20: Trigger (this questionwas askedadditionally to Q. 20) 1= Trauma or Stress 2= Bereavement 3= Stroke 4= Accident (RTA / fall, etc) 5= Medical (or Dental) Operation 6= Alcohol or Drug Induced 7= Other neuro disorder 8= No definabletrigger (Unknown) 9= Pregnancy/ Changeof life 10 = Familial II= Childhood (type) disease(eg glandularfever) 12 = Industrial injury 13 = R.S.I. (Repetitive Strain Injury) 14 = Heart Attack 15 = Flu or Cortisone Injections 16 = Cancer/ Radiotherapy 17 = Action induced/ Task Specific

70

Dystonia -A comprehensiveand longitudinal study in the North East of England

217 fam

relatesto Q. 21: Family members I= Yes (possible) 2 =No (definite) 3= Adopted

218 who2

relatesto Q. 21: Which family members

Temp = Who

I

= Father 2 = Mother 3 = Brother 4 = Sister 5 = Paternalrelation 6 = Maternal relation 7 = Son 8 = Daughter 9 = Spouse(wife) 10 = Nephew or Niece 219 dist2 I= 2= 3= 4= 5= 6= 7= 8= 220 221 222 223

who3 dist3 who4 dist4

NB : There is no who I label who2 relatesto dist2 who3 relatesto dist3 who4 relatesto dist4

relatesto Q. 21: Distribution

Temp = Distribution

Focal dystonia Segmentaldystonia Multi-focal dystonia Generaliseddystonia Hen-&dystonia Undefined spasms Other neuro disorder Undiagnosedspasms relatesto relatesto relatesto relatesto

Q. 21: Which family Q. 21: Distribution Q. 21: Which family Q. 21: Distribution

Template= Who Template= Dist Template= Who Template= Dist

$famtot Total of Family Members - variableswho2, who3 and who4 Multiple ResponseSet - Range I to 10 $disttot Total of Family Distribution - variablesdist2, dist3 and dist4 Multiple ResponseSet - Range I to 8 224 medl

relatesto Q. 22 & 26: Current Medication

BNF pageNo. No. 22: 1991

100 = Drugs which are Directly related to dystonia 101 = 102 = 103 = 104 =

Amanatadine (Symmetrel, Mantadine) Baclofen (Lioresal) Benzhexol (Artane, Broflex) Benztropine (Cogentin)

179 342 181 180

71

Dystonia -A comprehensiveand longitudinal study,in the North East of England

105 = Bromocriptine (Paroldel) 106= CannabisResin (not Nabilone - Cesamet) 107 = Chlorpromazine(Largactil) 108 = Clonazeparn(Rivotril) 109 = Co-beneldopa(Maclopar) 110 = Co-careldopa(Sinemet) 111 = Dantrolene(Dantrium) 112 = Diazepam(Valium, Stesolid,Diazemuls) 113 = Haloperidol (Serenace,Haldol, Dozic) 114 = Levodopa (Brocadopa,Larodopa) 115 = Lysuride Maleate (Revanil) 116= Methixene (Maleate) (Tremonil) 117 = Orphenedrine(Biorphen, Disipel) 118 = Pimozide (Orap) 119 = Primidone 120 = Selegiline(Eldepryl) 121 = Tetrabenazine(Nitoman) 122 = Thioridazine (Melleril) 123 = Trifluperazine (Stelazine)

ISO xxx 157 134 173 179 179 343 343 182 178 180 181 181 138 182 180 182 139 139

200 = Drugs which are Indirectly related to dystonia 201 = Amitripyline (Lentizol, Tryptizol)

144

202 = 203 = 204 = 205 = 206 = 207 = 208 = 209 = 210 = 211 = 212 = 213 = 214 = 215 = 216 = 217 = 218 = 219 = 220 = 221 = 222 = 223 = 224 = 225 = 226 = 227 =

328 181 165 172 146 130 176 131 130 146 161 162 162 161 151 330 331 166 167 94 146 146 162 344 331 151

Benorylate (Benoral) Biperiden (Akineton) Buprenorphine (Temgesic) Carbarnazepine (Tegretol) Clonýprarnine (Anafranil) Chlordiazepoxide (Librium) Chlormethiazole (Herninervin) Chlormezanone Clobazarn (Frisium) Clon-ýiprarnineHydrochloride (Anafranil) Co-codamol (Paracodomal) Co-codaprin Co-dydramol Co-proxamol (Distalgesic) Dexamphetan-line (Dexedrine) Diclofenac (Voltarol) Diflunisal (Dolobid) Dihydrocodeine (DF-I 18) Dipipanone Hydrochloride (Diconal) Dipyridamole (Persantin) Dothiepin (Prothiaden) Doxepin (Sinequan) Equagesic (not NHS) Feldene Gel Flurbiprofen (Froben) Fluvoxamine (Faverin)

72

Dystonia -A comprehensiveand longitudinal study in the North East of England

228 = Hyoscine (Scopodrem) 229 = Hypromellose (eye drops, Isopto) 23 0= Ibuprofen (Nurofen Brufen, Fenbid) , 231 = lmipran-ýineHydrochloride (Tofranil) 232 = Indomethacin 233 = Lofepramine (Gamanil) 234 = Lorazeparn 235 = Lormetazeparn 236 = Methadone Hydrochloride 237 = Naftidrofuryl (Praxilene) 238 = Naproxen (Naprosyn, Nycopren, Synflex) 239 = Nitrazepam 240 = Paracetamol. (Calpol, Disprol, Paldesic) 241 = Parafon (Cilag -in KSA) 242 = Paroxetine (Seroxat) 243 = Pergolide 244 = Phenytoin (Epanutin) 245 = Piroxicarn (Feldene) 246 = Prirnidone (Mysoline) 247 = Procyclidine (Arpicolin, Kemadrin) 248 = Propranolol (Inderal) 249 = Prozac (Fluoxetine Hydrochloride) 250 = Remoxipride (Roxiam) - withdrawn 251 = Risperidone (replacement Feb '94) 252 = Solpadeine (not NHS) 253 = Solpadol (Tylex) 254 = Sodium Valproate (Epilim) 255 = Sulpiride (Dolmatil, Sulpitil) 256 = Temazeparn 257 = Tiaprofenic Acid (Surgam) 258 = Trimipramine (Surmontil) 259 = Tryptophan 260 = Zopiclone (Zimovane)

156 355 330 146 331 147 131 127 183 87 333 126 160 new 151 xii 174 333 174 181 67 150 XII new 162 163 175 139 127 334 147 150 129

300 = Drugs wMch are Not related to dystonia 301 = Acebutolol (Sectral) 302 = 303 = 304 = 305 = 306 = 307 = 308 = 309 = 3 10 = 311 = 312 = 313 =

Alverine Citrate (Spasmonal) Aminophylline Amlodipine Besylate (Istin) Amoxy'cillin (Almodan) Antihistamines Aspirin Atenolol (Tenormin, Kalten, Tenio Beclomethasone, (Beconase, Becotide) Bendrofluazide (thiazide diuretic) Betahistine Hydrochloride (Sere) Betaxolol Hydrochloride (eye drops) Bismuth Chelate (De-nol)

68 30 109. 83 191 360 159 68 360/111 54 155 353 34

73

Dystonia -A comprehensiveand longitudinal study in the North East of England

314 = Bisoprolol Furnarate(Monocor) 315 = BromphenirarnineMaleate (Dimotane) 316 = Budesonide(Pulrnicort) 317 = Chlorambucil(Leukeran) 318 = ChlorpheniramineMaleate (Piriton) 319 = Cholestyramine(Questran) 320 = Clonidine (Dixarit) 321 = Co-codamol 322 = Co-codaprin 323 = CodeinePhosphate 324 = Co-dydramol 325 = Co-proxamol (Distalgesic) 326 = Cimetidine(Dyspamet,Tagamet) 327 = Cinnarizine(Stugeron) 328 = Cyclosporin (Sandimmun) 329 = CyproteroneAcetate (Dianette) 330 = Cytameninjections 331 = Danazol (Danol) 332 = Diclofenac (Voltarol) 333 = Digoxin (Lanoxin) 334 = Diltiazem Hydrochloride (Tildiem) 335 = Disodium Etidronate (Didronel) 336 = Diuretics (Dyazide, Frusene) 337 = Diuretics (Burinex, Neo-NaClex, Lasix) 338 = Domperidone(Motilium) 339 = Enalapril (Innovace) 340 = Expectorants(various) 341 = Fenbufen(Lederfed) 342 = Ferrous Gluconate/Sulphate(Ferrocontin) 343 = Folic Acid 344 = Frebusin 345 = Frusenýde(Lasix) 346 = Gaviscon(Algicon) 347 = Glibenclaminde 348 = Glyceryl Trinitrate (Suscard,Nitro-lin) 349 = HRT (General) 350 Hydroflumethiazide(Hydrenox) 351 Hypromellose(eye drops, Isopto) 352 Ibuprofen (Nurofen, Brufen, Fenbid) 353 Indapamide(Natrilix) 354 Indomethacin(Indocid-R) 355 IsosorbideMononitrate (Elanten,Ismo) 356 Ispaghula.Husk (Isogel,Metamucil,Fybogel) 357 Itraconazole(Sporanox) 358 Ketoprofen (Oruvail) 359 Lactulose 360 Lisinopril (Carace,Zesizil) 361 Loperamide(Imodium) 362 MaxiJul

68 115 112 284 115 97 170 161 162 37 162 161 32 155 291 388 302 264 330 52 83 261 58/59 60 155 77 123 331 298 302 504 56 27 238 80 250 55 355 330 55 331 82 41 220 332 45 78 37 506

74

Dystonia -A comprehensiveand longitudinal studý,in the North East of England

363 = MefenamicAcid (Ponstan) 364 = Mesalazine(Asacol) 365 = Metformin Hydrochloride 366 = Methotrexate Sodium (Arthitrex) 367 = MetoclopramideHydrochloride (Maxolon) 368 = Metopropol Tartrate (Betaloc, Lopresor) 369 = NEgraleve(Nfigravess,Paramax) 370 = Naproxen (Naprosyn,Nycopren, Synflex) 371 = Neomycin Sulphate(Otomize) 372 = Nifedipine (Adalat, Coracten) 373 = Norethiseterone(Primolut N) HRT 374 = Oestrogens(Premarin,Prempak) 375 = Omeprazole(Losec) 376 = Oxazepam 377 = Oxitropium Bromide (Oxivent) 378 = Oxprenolol (Trasidrex) 379 = Oxybutynin Hydrochloride (Ditropan) 380 = Oxytetracycline 381 = Paracetamol(Calpol, Disprol, Paldesic) 382 = Penicillin 383 = Phenylbutazone(Butacote) 384 = Philocarpine(eye drops) 385 = Pindocol (Viskew) 386 = Pizotifen (Sanomigran) 387 = Pravastatin(Lipostat) 388 = Prednisolone(Enteric) 389 = Prochlorperazine(Stemetil) 390 = Propranolol (Inderal) 391 = Quinine sulphate 392 = Ranitidine (Zantac) 393 = Salbutamol(Ventolin) 394 = Senna(Manevac, Senokot) 395 = Sertaline(Lustral) 396 = Simvastatin(Zocor) 397 = Sodium Valproate (Epilim) 398 = Sotalol Hydrochloride (Sotacom) 399 = Steroids(Nandrolone)

332 39 239 ABPI 156 69 169 333 358 84 255 252 35 131 107 70 279 202 160 188 333 352 71 170 99 248 158 67 342 34 103 43 151 100 175 71 256

400 = Drugs which are Not related to dystonia 400 = 401 = 402 = 403 = 404 = 405 = 406 = 407 = 408 = 409 =

Sucralfate (Antepsin) Sulphasalazine (Salazopyrin) Sumatriptin (In-ýigran- injection) Tamoxifen (Nolvadex) Terbutaline Sulphate (Bricanyl) Tenoxicam (Modiflex) Testosterone (Restandol, Virormone) Theophycline (Slo-phyllin) Thymoxamine (Opilon) Thyroxine (Eltroxin) [n&rograms]

35 39 ABPI 296 104 334 255 109 86 242

75

Dystonia -A comprehensiveand longitudinal study in the North East of England

4 10 = Tiaproferýc Acid (Surgam) 411 = Timolol Maleate (Timoptol) 412 = Verapamil (Berkatens,Cordilox, Securon) 413 = Vitanýins 414 = Warfarin (Marevan) 415 = Zopiclone (Zimovane) 225 dpdl

relatesto Q. 22/26: Dosage (mg) per day

226 whenI

relatesto when the medicationis taken I= Once a day 2= Twice a day 3=3xa day 4=4xa day 5= At night 6= As required 7= Taken weekly 8= Taken monthly 9= Injections

334 354 83 320 93 129

(od) (bd) (tds) (Qds) (nocte) (Prm)

(Prm)

227 med2

relatesto Q. 22 & 26: Medication

Template= Med

228 dpd2

relatesto Q. 22 & 26: Dosage (mg) per day

Template= Dose

229 when2 relatesto when the medicationis taken

Template= When

230 med3

relatesto Q. 22 & 26: Medication

Template= Med

231 dpd3

relatesto Q. 22 & 26: Dosage (mg) per day

Template= Dose

232 med4

relatesto Q. 22 & 26: Medication

Template= Med

233 dpd4

relatesto Q. 22 & 26: Dosage (mg) per day

Template= Dose

234 med5

relatesto Q. 22 & 26: Medication

Template= Med

235 dpd5

relatesto Q. 22 & 26: Dosage (mg) per day

Template= Dose

$medtot Total medications - variablesmedl, med2, med3, med4 and med5 Multiple ResponseSet - Range 101 to 415 $dpdtot Total of dosage per day - variablesdpdI, dpd2, dpd3, dpd4 & dpd5 Multiple ResponseSet - Range I to 988 SwhentotTotal of when taken - variableswhenI and when2 Multiple ResponseSet - Range I to 9

76

Dystonia -A comprehensiveand longitudinal study in the North East of England

236 pay

relatesto Q. 22: Pay for medication I= Private Prescription 2= Patient paid NHS 3= PrescribedFOC (to patient)

237 prevI

relatesto Q. 24: Previous treatments

Template= Prev.

I= Drugs / Medication 2= Surgery 3= Psychiatry 4= Osteopathy 5= Chiropractic 6= Biofeedback 7= Psychologist(Counselling StressClinic) Physiotherapy 8 Aromatherapy 9 10 Acupuncture II =Yoga 12 =Meditation 13 = Hypnotherapy 14 = AlexanderTechnique 15 = Wearing a collar 16 = Heat Treatment(Electric Treatment TENS Machine) 17 = SpeechTherapy 18 = Healing Hands (Spiritualism) 19 = Reflexology 20 = OrthopaedicSurgery 21 = Manipulation (under anaesthetic) 22 = Homeopathy(Herbal remedies) 23 = Rheumatology 24 = Relaxationtechnique 25 = Cortisone injections (or Lignocaine) 26 = Phenol injections 27 = Immunology 28 = Ophthalmology 29 = Hydrotherapy 238 prev2

relatesto Q. 24: Previous treatments

239 prev3

relatesto Q. 24: Previous treatments

240 preA

relatesto Q. 24: Previous treatments

241 prev5

relatesto Q. 24: Previous treatments

Template= Prev.

$prevtot Total of previous treatments - variablesprevI to prev5 inclusive Multiple ResponseSet - Range I to 29

77

Dystonia -A comprehensiveand longitudinal study in the North East of England

242 septl

relatesto Q. 25: Side effects previous treatments Template= Side I= Felt ill / more pain 2= Increased spasms 3= Lack muscle control 4= Nausea 5= Drowsiness 6= Flu like symptoms 7= Dysphagia 8= Feeding by tube 9= Headaches 10 = Tickling throat II= Coughing 12 = Sneezing 13 = Phlegm 14 = Dry eyes 15 = Depression 16 = Ptosis 17 = Difficulty walking 18 = Double vision (blurred vision) 19 = Hallucinations (Confused) 20 = Constipation 21 = Loss of weight 22 = Dry mouth / throat 23 = Elation 24 = Tiredness / Lethargy 25 = Watery eyes 26 = Skin rash 27 = Increased weight 28 = Bruising / bleeding 29 = Dizziness 30 = Memory Loss 31 = Allergic reaction 32 = Insomnia 33 = Addiction 34 = Hospitalisation

243 sept2

relatesto Q. 25: Side effects (prev)

Template= Side

$septtot Total side effects from previous treatments variable septl, sept2 Multiple ResponseSet - Range I to 34

78

Dystonia -A comprehensiveand longitudinal study in the North East of England

244

last I

relatesto Q. 25: How long it lasts

Template= Last

I= One day 2=A couple of days 3= About a week 4= Over a week 5=A month or over 6= Until stoppedtaking 7= Undefinedperiod 8=2-3 weeks 9= Up to 2 months 245 coml

relatesto Q. 00: Co-morbidity I

Info ftom CAF

100 = Co-morbidity Directly related to dystonia Temp = Comorbidity 101= Anoxic Brain Damage 102 = BET (Benign EssentialTremor) 103 = Chiari Malformation 104 = Chorea 105 = CerebralAneurysm (or Carcinoma) 106 = CerebralPalsy 107 = Death (not related to dystonia) 108 = Gilles de Tourette (Syndrome) 109 = Head Injury I 10 = Leigh's Disease III= Meningoencephalitis/ Encephalomyelitis 112 = Multiple Sclerosis 113 = MSA (Multiple SystemAtrophy) 114 = Myoclonus 115 = Parkinson'sDisease 116 = PneumococcalMeningitis 117 = Shy Drager Syndrome 118 = Stroke 119 = Spasticity (SubarachinoidHaemorrhage) 120 = Tardive Dyskinesia 200 = Comorbidity Indirectly related to dystonia 201 = Agoraphobia 202 = Alcoholism 203 = Anorexia Nervosa 204 = Anxiety Neurosis 205 = Bell's Palsy 206 = CerebellaAtaxia 207 = Depression 208 = Drug Addiction 209 = Drug InducedPD 2 10 = Epilepsy 79

Dystonia -A comprehensiveand longitudinal study in the North East of England

211 =Insomnia

212 = Learning Difficulties 213 = Manic Depressive(Psy6hotic) 214 = PD (as well as dystonia) 215 = PersonalityDisorder 216 = Post 'psychosurgery' (thalomotomy) 217 = Schizophrenia(psychosis) 218 = Spondylosis,Cervical 300 = Co-morbidity Not related to dystonia 301 = Acne 302 = Addison'sDisease 303 = Alcoholism 304 = Allergic Rhinitis 305 = Alzheimer'sDisease 306 = Anaerffla(Iron Deficiency) 307 = Angina 308 = Anxiety Neurosis 309 = Arthritis (unspecific or osteo) 3 10 = Asthma 3 11 = Atria Fibrillation 312 = Breast Carcinoma 313 = Bronchial Carcinoma 314 = Bronchitis (Chronic) 315 = CardiacFailure 316 = CerebrovascularDis(ease) 317 = Cervical Myelopathy 318 = Cholecystectomy 319 = Chronic Fatigue Syndrome(ME) 320 = Constipation 321 = CTS (Carpel Tunnel Syndrome) 322 = Deaffiess 323 = Depression 324 = Diabetes 325 = Diverticular Disease 326 = Endometriosis 327 = Epilepsy 328 = Glaucoma(plus I caseof Iritis) 329 = Gout 330 = Hay Fever (incl Urticaria) 331 = Hernia (Oesophagealor Hiatus) 332 = Hepatitis - Viral 333 = HRT (Hormone ReplacementTherapy) 334 = HypercholesterolAnaemia 335= Hypertension 336 = Hysterectomy 337 = IBS (Irritable Bowel Syndrome) 338 = Inflammatory Bowel Disease 80

Dystonia -A comprehensiveand longitudinal study in the North East of England

339 = Insomnia 340 = Ischaemic Heart Disease 341 = Kidney Transplant (or Renal problems) 342 = Leg Cramps 343 = Lumbar pain 344 = Lymphocytic Leukaernia (Chronic) 345 = Lymphoedema 346 = Lymphoma 347 = Muscular Degeneration 348 = Meniere's (and / or Tinnitus including Vertigo) 349 = Menstrual Symptoms (Pain) 350= Migraine 351 = Osteoporosis 352 = Paget's Disease 353 = Parotid Tumour (Adenoma) 354 = Peripheral Neuropathy 355 = Peripheral Vascular Disease 356 = Pernicious Anaemia 357 = PMT (Pre-menstrual Tension) 358 = Polymyalgia Rheumatica 359 = Progressive Systemic Sclerosis 360 = Prolactinomaectomy 361 = Prostastic Carcinoma 362 = Psoriasis 363 = Raynaud's Syndrome 364 = Rheumatoid Arthritis 365 = Sandhoff s Disease 366 = Sick Sinus Syndrome 367 = Sinusitis 368 = Surgery, Knee or Hip 369 = Splenectomy 370 = Thrush (Candidiasis) 371 = Thyroid Disease 372 = Lflcer (Peptic also Duodenal) 373 = Ulnar Nerve Palsy 374 = Valvular Heart Disease

246 com2 247 com3 248 com4

relatesto Q. 00: Co-morbidity 2 relatesto Q. 00: Co-morbidity 3 relatesto Q. 00: Co-morbidity 4

Temp = Comorbidity Temp = Comorbidity Temp = Comorbidity

$comtot Total co-morbidity - variable comI, com2, com3 and com4 Multiple ResponseSet - Range 101 to 374 249 bef

relatesto Q. 29: Bot Tox before No of times before the interview or the start of the research 0= Never beeninjected before

81

Dystonia -A comprehensiveand longitudinal study in the North East of England

250 251 252 253 254 255 256

inj I inj2 inj3 inj4 iqJ5 iqJ6 iqJ7

Injections from beginning to 31.03.94 01.04.94- 31.03.95 01.04.95- 31.03.96 01.04.96- 30.11.96 04.12.96- 25.03.97 01.04.97- 25.06.97 01.07.97- 31.12.98

ffom hospital records from files, odd missingdata ftom hospital records from files, odd missingdata ftorn hospital records from hospital records from hospital records

257 curr

relatesto Q. 29: Current No. Bot Tox No of times up to 01.01.99 - from clinical records up to subjectno 885, from subjectno 885 onwards the numberof times at date of interview. -

258 bodl

relatesto Q. 29: Where in body I I= 2= 3= 4= 5= 6= 7= 8= 9=

259 bod2

Template= Bodyl

Eye Muscles FaceMuscles Neck or Shoulder(Muscles) Throat / Larynx Arm Hand Leg Foot Abdomen (Trunk or Back) Mouth Jaw

relatesto Q. 29: Where in body 2

Template= Bodyl

$bodtot Total sites in body - variable bodl. and bod2 Multiple ResponseSet - Range I to 9 260 sebtl

relatesto Q. 29: Side effects (Bot Tox) I Samecodesused as in # 242 'septl'

Temp= Side

261 sebt2

relatesto Q. 29: Side effects (Bot Tox) 2

Temp = Side

SsebttotTotal Side Effects (Bot Tox) - variable sebtl and sebt2 Multiple ResponseSet - Range I to 34 262 last2

relatesto Q. 29: How long it lasts

Temp = Last

I= One day 2=A couple of days 3= About a week 4= Over a week 5=A month or over 6= Until treatmentstop 7= Undefinedperiod 8=2-3 weeks 9= Up to 2 months

82

Dystonia -A comprehensiveand longitudinal study in the North East of England

TDS Questionnaire: Q're No. 21 263 talk

relatesto Q. 30: Talked to others I= 2= 3= 4= 5= 6= 7=

264 what

Never Rarely Occasionally Often Frequently Once only One persononly

relatesto Q. 3 1: Under what circumstances I= At BT clinic 2 = In hospital 3 = At home 4 = On the phone 5 = At TDS meetings 6 = Never talked 7 = At work

265 counI

relatesto Q. 32: Counselling I= Yes 2=No 3= Don't know

266

coun2

TDS Counselling Service used as of 31.12.98

I= 2= 3= 4= 267 when

Full counselling With family members One-off sessiononly One-off with family

relatesto Q. 32: When counselling I= 2= 3= 4= 5=

When first diagnosed If required / needed When required/ need Not required by me Don't know

83

Dystonia -A comprehensiveand longitudinal stud),in the North East of England

268 whom

relatesto Q. 32: Who counsels I= 2= 3= 4= 5= 6= 7= 8=

269 tds

By the doctor (neurologist) By a nurse (practitioner) By a social worker (Social Services) By someonewho knows (about it) By someonewith it By someonein NHS By someonefrom TDS Don't know (not answeredpreviously)

relatesto Q. 33: Heard of TDS I= Another member 2= Relative or ftiend 3= National T. V. or Radio 4= Newspaper/ magazine 5= Doctor or Hospital or Clinic 6= Thru this research 7= Leaflets / Adverts (North East only) 8= Can't remember 9= Disability Group 10 = Library or CAB (Citizens Advice Bureau) 11 = TTTV I in 1994 12 = TTTV 2 in 1997 13 = BBC TV (NE) in 1997

270 mern

relatesto Q. 34: Member of TDS I =Yes 2 =No 3= No longer a member(dropped out) 4= Joined since study (interviewed) 5= Member of MSS, PDS (etc) 6= Deceasedmember 7= Dropped out and re-joined 8= Joinedby RC 1 9= Joinedfor WBS 10= Joinedby RC 2

271 joinI

relatesto Q. 35: Year & Month when joined (or re-joined) From 8302 onwards only valid

272 join2

relatesto Q. 35: Yearjoined TDS From 83 to 99 only

273 numb

TDS Membership Number (from HQ records) 0= Number unknown

84

Dystonia -A comprehensiveand longitudinal study in the North East of England

274 why

relatesto Q. 35: Why joined TDS I= 2= 3= 4= 5= 6= 7= 8= 9=

275 most

relatesto Q. 36: Most useful aspect I= 2= 3= 4= 5= 6= 8=

276 shg

To find out more (about dystonia) To meet and talk (to others / support) To read the literature To attend TDS (meetings) To receivethe newsletter To get information (in general) To get answers(to my questions) Don't know To give support

The newsletter Information (in general) Getting answers(to my questions) Being part of SHG (activities) Contact with others (people) News about research Don't know

relatesto Q. 37: Self-Help Groups I =Teesside SHG 2 =Tyneside SHG 3 =Cumbria SHG 4= Yorkshire Area (Hull, Leeds, Sheffield,etc) 5= Member of another SHG 6= TDS member,not SHG 7= On mailing list only 8= No contact (requested) 9= Darlington SHG - 1998 10 = SunderlandSHG - 1998 11 = Durham SHG - 1998

277 get

relatesto Q. 38: Get most out of TDS I= Feelingof belonging 2= Sharingexperience 3= Talking to others 4= Social contact 5= Readingthe newsletter = Finding out others (about people) = More knowledge (about condition) = Don't know

85

Dystonia -A comprehensiveand longitudinal study in the North East of England

278 news

relatesto Q. 38: Newsletter I= 2= 3= 4= 5= 6= 7= 8= 9=

279 aiml

Medical articles Latest research Letters to Editor (other people) Contact addresses News - other groups Overseasinformation All of it Dodt know Can't read

relatesto Q. 39: National aim

Template= Aim

I= Contact with others (help and support) 2= Raising awareness(medical and public) 3= Raisingfunds 4= Finding a cure 5= Funding research 6= Giving information 7= Counselling/ Welfare 8= Don't know 9= Getting treatment 10 = DNP (more required) 280 aim2

relatesto Q. 39: Local aim

Template= Aim

86

Dystonia -A comprehensiveand longitudinal study in the North East of England

Clinical FeaturesQuestionnaire: Q're No. 30 This questionnaireon the "Clinical Featuresof Dystonia" is divided into five parts :1. Contact with Medical Services 2. Clinical Featuresof your Dystonia 3. Nature of your Accommodation 4. Questionnaireon Torticollis 5. GesteAntagoniste

Questions 01 13 to Questions 14 20 to Questions 21 23 to Questions 24 25 to Questions 32 26 to -

281 date30 Date of Qre 30

dd.mm.yy 8

282 onset2 relatesto Q. 01: Year of onset from the questionnaire 24 = 1924, then includes every year to 98 = 1998 283 diff2

Difference between onsetl and onset 2 (in years) Onset I date was obtainedby interview (records) Onset2 date was obtainedby questionnaire bias shows a questionnaire -5 0 showsno difference +5 showsan interview bias

Compute diff2 = onsetI- onset2. Execute. 284 told

relatesto Q. 02: Told of diagnosis (year) Different to being diagnosed,ie being told - compareto # 213 'diag'

285 M6

Difference between being diagnosed and being told before being 18 18 told = years before being told years -8=8 before being told years -3=3 0= Diagnosedand told in sameyear I=I years error 3=3 years error 6=6 years error 92 = Never told (of diagnosis)since 1992 94 = Never told (of diagnosis)since 1994 96 = Never told (of diagnosis)since 1996

Compute diff6 = diag -told. Execute.

87

Dystonia -A comprehensiveand longitudinal study in the North East of England

286 del

relatesto Q 03: Delay in diagnosis I= Dr, unawareof condition / lack of knowledge 2= NEs-diagnosed 3= Nfis-diagnosed& treated for ....... 4= Mis-diagnosedCP/N4S/PD 5= Did not seekadvice 6= Diagnosedother (neuro disorder) 7= Unknown Cause/ Reason 8= No (long) delay (NHS waiting list) 9= Waiting for appointment(to be diagnosed) 10 = Went into remission

287 doc

relatesto Q 04: Who diagnosed (dystonia) I= Own G.P. 2= Hospital Dr / Neuro. 3= Private Dr / Neuro. 4= Psychiatrist 5= ENT Surgeon 6= Neuro Surgeon 7= Ophthalmologist 8= Psychotherapist 9= Self-diagnosed(family) 10 = Psychopharmacologist II= SpeechTherapist 12 = Rheumatologist 13 = Physiotherapist 14 = Thru' child I st 15 = Urologist

288 evntl

relatesto Q 05: Major event

Template= Yes / No

I =Yes 2 =No 289 evnt2

relatesto Q 05: Type of major event

Template = Event

I= Major headinjury 2= Other neuro disorder 3= CerebellaAtaxia 4= Minor headinjury 5= Stresscausedby ...... 6= Fall 7= Stroke / Cere/ SAH 8= Non related condition 9= Bereavement 10 = Surgery II= Divorce 88

Dystonia -A comprehensiveand longitudinal study in the North East of England

12 = Accident (work / home) 13 = SevereMigraines 14 = RTA (Road Traffic Accident) 15 = Menopause 16 = RSI (Repetitive Strain Injury) 17 = Heart Attack 18 = Anxiety 19 = Measles 20 = Pregnancy(incl one still birth) 21 = Exhaust/ stressat work 22 = Drug Overdose(Tranquillisers) 23 = Flu Injections 24 = Gaveup smoking 25 = W.W. II (difficult birth bomb blast) 26 = Schizophrenia 27 = Alcoholism 28 = Hereditary / familial 29 = Don't know 30 = Chemicalexposure 31 = Physicalstrain 32 = Trappednerve 33 = Born.with it 34 = Puberty 35 = Use of drugs 36 = Slipped disc 37 = Viral infection 290

caus

relatesto Q 06: What caused it (your dystonia) Template= Event

291

mentl

relatesto Q 07: Suggest it was mental

Temp = Yes / No

292

gp

relatesto Q 08: G.P. knows enough

Temp = Yes / No

293

drug

relatesto Q 09: Drugs - Yr started 0= Unknown year

294

effl

relatesto Q 09: Effect of drugs

Template= Effect

I= Better 2= Unchanged 3= Worse 295 296 297 298 299 300 301

surg efl2 physio eM osteo eff4 chiro

relatesto Q 09: Surgery - Yr started relatesto Q 09: Effect of surgery relatesto Q 09: Physiotherapy - Yr started relatesto Q 09: Effect of physiotherapy relatesto Q 09: Osteopathy - Yr started relatesto Q 09: Effect of osteopathy relatesto Q 09: Chiropractic - Yr started

Template= Year Template= Effect

89

Dystonia -A comprehensiveand longitudinal study in the North East of England

302 303 304 305 306 307 308 309 310 311 312 313 314 315 316317 318 319 320

Effect Template Q Effect 09: = to or chiropractic relates eff5 Template Year Yr Q biof Biofeedback 09: = to started relates biofeedback Q Effect 09: to of relates eff6 Yr Counselling Q 09: to started couns relates relatesto Q 09: Effect of counselling eff7 Yr Q 09: Psychotherapy to started psycho relates eff8 relatesto Q 09: Effect of psychotherapy acup relatesto Q 09: Acupuncture - Yr started relatesto Q 09: Effect of acupuncture eff9 Yr Q Yoga 09: to started relates yoga Effect Q 09: 0 to of yoga effl. relates Yr Q 09: Meditation to started medi relates relatesto Q 09: Effect of medication effl. I Yr Q Hypnotherapy hypn 09: to started relates relatesto Q 09: Effect of. hypnotherapy effl.2 Yr Tech Q Alexander 09: to started relates alex Technique Alexander Q Effect 09: 3 to of effl. relates botox relatesto Q 09: Bot Tox therapy - Yr started eff14 relatesto Q 09: Effect of Bot Tox therapy 0= I= 2= 3=

Too early to define Better Unchanged Worse

321 otherl. relatesto Q 09: Other treatments - Yr started 322 other2 relatesto Q 09: Type of treatment I= Faith Healing (2 x Spirit; 2x Faith; Ix Prayer) 2= Personal.Method (I x Drugs; 2x Unknown, Ix Routine 3= Collar (Wearing) and Ix Champissape??) 4= Homeopathy 5= Aromatherapy 6= Manipulation 7= SpeechTherapy 8= Herbal remedies 9= Reflexology 10 = Traction II= RelaxationTapes 12 = Heat / Elec Stimulation (I x Heat and Ix Elec) 13 = TENS Machine 323 efn 5

relatesto Q 99: Effect of other treatments

Template= Effect

324 maj

illness (physical) Major Q 10: to relates

Template= Yes / No

90

Dystonia -A comprehensiveand longitudinal study in the North East of England

325

physl

relates to Q 10: Type of physical illness I

I= Achilles Tendon 2= Adrenal glands (removed) 3= Alcoholism 4= Appendicitis (ectomy) 5= Arthritis (unspecifiedand osteo) 6= Asthma 7= Bomb Blast 8= Bones - fractures, etc 9= Brain - operation 10 = Brain Tumour II =Bronchitis 12 = Cancer(unspecified) 13 = CardiovascularDis. (inc Angina, Bypass,etc) 14 = CerebellaAtaxia 15 = Cervical Spondylosis 16 = CP 17 = Crohn'sDisease 18 = Dental Operation 19 = Diphtheria 20 = Diverticulitis 21 = Ectopic Pregnancy 22 = Endometriosis 23 = Epilepsy 24 = Eyelid surgery 25 = Gall stones / bladder (removed) 26 = Gastro-enteritis 27 = Glandular Fever 28 = Glaucoma 29 = Growth removed (various) 30 = Guillam Barre Syndrome 31 = Heart attack 32 = Hepatitis 33 = Hernia - Hiatus 34 = Hip Replacement 35 = Hydrocephalus 36 = Hypertension 37 = Hysterectomy (incl Tubal Tic) 38 = Inf. Bowel Disease 39 = Infantile Paralysis (Polio) 40 = Kidney Transplant (stones) 41 = Knee (Joint) Operation 42 = Leg / Arm / Hand Operation 43 = Malnutrition 44 = Mastoid Operation 45 = Meningitis 46 = Migraine 47 = MS

91

Dystonia -A comprehensiveand longitudinal study in the North East of England

48 = Neck - Operation (incl I-IFS operation) 49 = Osteoporosis(brittle bones) 50 = Otitis Media 51 = Paralysed- both legs 52 = PD 53 = Pneumonia 54 = ProlapseOperation 55 = Prostateremoved 56 = RheumaticFever 57 = RTA (incl stabbed, ladder fall, assault, accident) 58 = SAH

59 = Sarcoidproblems 60 = ScarletFever 61 = Shingles 62 = Skin Complaint 63 = SlippedDisc 64 = Stroke 65 = Thyroid (ectomy) 66 = TB 67 = Throat Cancer 68 = Throat Operation 69 = Tinnitus / Vertigo 70 = Tonsillitis 71 = Tracheotomy 72 = Ulcer 73 = UnspecifiedOperation 326 yearl.

relatesto Q 10: Year of InIness1

327 328 329 330 331 332

relates to relates to relates to relates to relates to relates to

phys2 year2 phys3 year3 phys4 year4

9 Q Q Q Q Q

10: Type 10: Year 10: Type 10: Year 10: Type 10: Year

of of of of of of

physical illness 2 Iffiness 2 physical illness 3 Iffiness 3 physical illness 4 Illness 4

Template= Year Template = Physical Template = Year, etc

$phystot Total physical illnesses- variable physl to phys4 inclusive Multiple ResponseSet - Range I to 73 $yrltot Total Year of Onset - variable yearl to year4 inclusive Multiple ResponseSet - Range0 to 98 333 ment2 relatesto Q 11: Psychiatric problems ?

Temp = Yes / No

92

Dystonia -A comprehensiveand longitudinal study in the North East of England

334 psycl

relatesto Q 11: Type of psychiatric problem I

Temp = Psychiatric

I= Depression 2= Anorexia 3= Anxiety 4= Nervous Breakdown' 5= Tremor (of head) 6= ImaginedDystonia 7= Paranoia 8= Schizophrenia 9= Amnesia 10 = Agoraphobia II= Loss of speech 12 = Epilepsy 13 = Panic Attacks 14 = Depressionand Anxiety 15 = Alcoholism 16 = ObsessiveNeurosis 335 year5

relatesto Q 11: Year of illness 5

Template= Year

336 tretl

relatesto Q 11: Treatment received I

Template Treat.

I= Medication / Drugs 2= Surgery 3= Physiotherapy 4= Osteopathy 5= Chiropractic 6= Biofeedback 7= Counselling 8= Psychotherapy 9= Acupuncture 10 = Yoga II =Meditation 12 = Hypnotherapy 13 = AlexanderTechnique 14 = Bot Tox Therapy 15 = Shock treatment (ECT) 16 = Injections 17 = SpeechTherapy 337 psyc2 338 year6 339 tret2

relatesto Q 11: Type of psychiatric problem 2 Temp = Psychiatric Temp = Year relatesto Q 11: Year of illness 6 Temp = Treatment relatesto Q11: Treatment received 2

SpsyctotTotal psychiatric illnesses- variable pyscl and pysc2 Multiple ResponseSet - Range I to 16

93

Dystonia -A comprehensiveand longitudinal study in the North East of England

$trettot Total psychiatric treatment - variable tret I and tret2 Multiple Response Set - Range I to 17

Syrstot Total date (year) of illnesses- variableyear5 and year6 Multiple ResponseSet - Range0 to 98 340 anti 341 anxi

relatesto Q 12: Antidepressants relatesto Q 13: Control of anxiety

342 343 344 345 346 347 348 349 350 351 352 353 354 355 356 357 358 359 360 361 362 363 364 365

Template Year relatesto Q 14: Year affected (Left eye) relatesto Q 14: Year affected (Right eye) relatesto Q 14: Year affected (Both eyes) relatesto Q 14: Year affected (R side of face) relatesto Q 14: Year affected (L side of face) relatesto Q 14: Year affected (Both sides of face) relatesto Q 14: Year affected (mouth) relatesto Q 14: Year affected (jaw) relatesto Q 14: Year affected (tongue) relatesto Q 14: Year affected (vocal chords) relatesto Q 14: Year affected (speech) relatesto Q 14: Year affected (right arm) relatesto Q 14: Year affected (right hand) relatesto Q 14: Year affected (left arm) relatesto Q 14: Year affected (left hand) relatesto Q 14: Year affected (right hand) relatesto Q 14: Year affected (left hand) relatesto Q 14: Affected only when writing ? Template Yes No relatesto Q 14: Year affected (trunk) relatesto Q 14: Year affected (right leg) relatesto Q 14: Year affected (right foot) relatesto Q 14: Year affected (left leg) relatesto Q 14: Year affected (left foot) add variable : Year affected (neck) - deducedfrom Q'res

eyel eyer eyes facer facel faces mouth jaw tongue vocal speech arnir handr arml handl writl wfit2 writ3 trunk legr footr legl fbotl neck

Template= Yes No Template= Yes No

366 movel. relatesto Q 15: Involuntary movements

Template= Yes / No

367 move2 relatesto Q 15: Type of movement 2

Temp = Movement

I= 2= 3= 4= 5= 6= 7= 8= 9=

Regular trembling Quick jerking Slight twitching Slow pulling / turning Eyelids shut Occasionaltrembling Cramps/ Cramping Nfixture of I to 4 Rhythmical (continuous)

94

Dystonia -A comprehensiveand longitudinal study in the North East of England

10 = Voice VvUspers II= Stiffhess(Spasticity / Rigidity) 12 = SuddenJerk (once only at a time) 13 = Action Induced/ Task Specific 368 move3 relatesto Q 15: Type of movement 2 369 move4 relatesto Q 15: Type of movement 3

Temp = Movement

$movetot Total types of movement - variable rnove2,rnove3 and rnove4 Multiple ResponseSet - Range I to 13 370 seve

relatesto Q 16: How severe is your dystonia 0= Not severeat all 10 = Very severe

371 contl

relatesto Q 17: Degree of control

Template= Control

0= No control at all 10 = Completecontrol 372 cont2

relatesto Q 18: Degree of control

Template= Control

373 pain3

relatesto Q 19: Pain at present

Template= Yes /No

374 pain4

relatesto Q 19: Where pain 1

Template= Body2

I= Eye Muscles 2= FaceMuscles 3= Neck Muscles 4= ShoulderMuscles 5= Arm Muscles 6= Hand Muscles 7= Leg Muscles 8= Foot Muscles 9= Trunk Muscles 10 = Back Muscles II =Mouth Muscles 12 = Jaw Muscles 13 = Head 14 = Throat / Larynx 375 376 377 378

pain5 pain6 pain7 pain8

relatesto Q relatesto Q relatesto Q relatesto Q

19: Where 19: Where 19: Where 19: Where

pain pain pain pain

2 3 4 5

Template = Body2

$paintot Total where pain - variable pain4 to paing inclusive Multiple ResponseSet - Range I to 13 95

Dystonia -A comprehensiveand longitudinal study in the North East of England

379 pain9

relatesto Q 19: Severity of pain 0= No pain 10 = Very severepain

380 painIO relatesto Q 19: How frequent pain I= Infrequently 2= Often 3= Continuously 381 sponI

relatesto Q 20: Spontaneous improvement

Template= Yes / No

382 spon2 relatesto Q 20: Year of improvement I

Template= Year

383 spon3 relatesto Q 20: How long

Template= Long

I=A few daysonly 2= Severalweeks 3=4 weeks 4= More than a month 5=6-8 weeks 6=2 months 7=3 months 8=6 monthsto a year 9= Over a year 10 =2 years II= Severalyears 12 =4 years 13 = Over 5 years 14 =6 years 15 =8 years 16 = 15 years 17 = 27 years 18 = Still in remission 384 spon4 relatesto Q 20: Partial or complete I I= Partial 2= Complete

385 spon5 relatesto Q 20: Year of improvement 2 386 spon6 relatesto Q 20: How long 387 spon7 relatesto Q 20: Partial or complete 2

Ternp Year Temp Long

$yeartot Total years of improvement - variable spon.2 and spon5 Multiple Response Set - Range 00 to 98

96

Dystonia -A comprehensiveand longitudinal study in the North East of England

$longtot Total how long - variable spon3and spon6 Multiple ResponseSet - Range I to 18 $parttot Total partial / complete - variable spon4and spon7 Multiple ResponseSet - Range I or 2 388

livel

relatesto Q 21: Who do you live with ? I= 2= 3= 4= 5= 6= 7=

389

live2

Alone With spouse With parents With children With sibling With aunt Girl / Boy Friend (Live in Partner)

relatesto Q 22: Type of accommodation I= House 2= Flat 3= Bungalow 4= Terraced Cottage 5=B&B 6= Prison 7= Residential Home

390

live3

for dystonia Q Suitable 23: to relates

391

live4

relatesto Q 23: Ways it does not meet your needs

Template= Yes/No

I= Stairs (too many or difficult) 2= No shower+ stairs (too many) 3= No WC downstairs 4= No help at home 5= More adaptationsneeded 6= Doors too small (for wheelchair) 7= House too big (to keep clean) 8= Outside Steps(Wheelchair) 9= Being adapted(lift / handrails,etc) now 10 = (haveto) SleepDownstairs

97

Dystonia -A comprehensiveand longitudinal study in the North East of England

Torticollis Questionnaire: Q're No 40 392 date40 Date of Qre 40

dd.mm.yy 8

393 pstnI

Numeric 4.0

relatesto Q 24: Position of head I= 2= 3= 4=

Chin turns to the side Ear tilts sidewaystowards shoulder Head bendsforward (chin pointing C, down) Headsbendsbackwards(chin pointing up)

394 pstn'-) relatesto Q 25: Direction of head 10 = Right turn or tilt II= Right turn 12 = Right tilt 20 = Left turn or tilt 21 =Left turn 22 =Left tilt 3= Backwards (chin pointing up) 4= Forwards (chin pointing down) 5= Right and backwards 6= Right and forwards 7= Left and backwards 8 =Left and forwards

395 gestl

relatesto Q 26: Geste Antagoniste

396 gest2

relatesto Q 26: Still effective ?

Temp = Yes / No

I =Yes 2=No 3= Not necessarynow (in remission) 397 gest3

relatesto Q 26: If No, how many years ? 0= Unknown no of years

398 gest4

relatesto Q 27: Describe in detail I= Written description (attached/ enclosed)

399 handl

relatesto Q 28: Which hand (do you use) ? NB : Other questionsare Left (1) then Right (2) I= Right hand 2= Left hand 3= Either (both) hand

98

Dystonia -A comprchcnsivc and longitudinal study in the North East of England

400 truel

relatesto Q 29: Where ? I= 2= 3= 4=

401 true2

relatesto Q 30: How I= 2= 3= 4=

402 true3

Front of my body Back of my body Side of face Different (on different) occasions

Lightly touch Pull hard Pushhard Different (on different) occasions

relatesto Q31: Moving when ? I Before 2 At the sametime 3 After 41 or 3 (sometimes)

403 best

relatesto Q 32: Feeling ? I= Being pulled 2= Being pushed 3= Both at times

99

Dystonia -A comprehensiveand longitudinal study in the North East of England

Living with Dystonia Questionnaire: Q're No. 50 This questionnaireis a compositeof 4 distinct questionnaires:1. A FunctionalDisability Questionnaire(FDQ): PagesI and 2 2. A Body ConceptScale(BCS) Pages3 and 4 3. The Beck DepressionInventory (BDI) Pages5,6 and 7 4. Rosenberg'sSelf-EsteemScale(SES) Page8 404 date5O Date of Qre 50 405 fdql

relatesto Q 01: Dressing (Self Care)

dd.mm.yy 8 Temp = Affected

0= Not applicable Not at all affected .1= 2= Mildly affected 3= Moderately affected 4= Severelyaffected 406 407 408 409 410 411 412 413 414 415 416 417 418 419 420 421 422 423 424

fdq2 fdq3 fdq4 fdq5 fdq6 fdq7 fdq8 fdq9 fdqlO fdql I fdql2 fdql3 fdql4 fdq15 fdql6 fdql7 fdql8 fdq19 fdq20

relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to relates to

Q 02: Housework (Physical) Q 03: Television (Leisure) Q 04: Running (Physical) Q 05: Transport (Social) Q 06: Writing (Leisure) Q 07: Conversation (Social) Q 08: Carrying (Physical) Q 09: Restaurants or pubs (Social) Q 10: Brushing teeth (Self Care) Q 11: Reading (Leisure) Q 12: Walking (Physical) Q 13: Intercourse (Physical) Q 14: Driving (Physical) Q 15: Washing (Self Care) Q 16: Eating (Social) Q 17: Dinner parties (Social) Q 18: Typing (Physical) Q 19: Hobbies (Physical) Q 20: Crossing roads (Social)

425 fdq21

face Self (if Care) Q 21: Shaving to male relates female Care) (if Self on make up or putting -

426 427 428 429 430 431

relatesto Q 22: Drinking (Social) relatesto Q 23: Riding a bicycle (Physical) relatesto Q 24: Theatre (Social) relatesto Q 25: Coordination (Self Care) relatesto Q 26: Sports (Physical) relatesto Q 27: Stairs (Physical)

fdq22 fdq23 fdq24 fdq25 fdq26 fdq27

100

Dystonia -A comprehensiveand longitudinal study in the North East of England

NB :3 subjectshad data removed,as they were incompleteand skewing scores. ESD No. 74 - variablefdq7 was removed. ESD No. 171 - variablesfdqI, fdq2 and fdq3 were removed. ESD No. 433 - variablesfdqI to fdql2 (inclusive) were removed. 432 fdqaII

FDQ - Total all scores

Computefdqall = fdql + fdq2 + fdq3 + fdq4 + fdq5 + fdq6 + fdq7 + fdqg + fdq9 + fdqlO + fdql I+ fdql2 + fdql3 + fdql4 + fdql5 + fdql6 + fdql7 + fdql8 + fdql9 fdq20 + fdq2l + fdq22 + fdq23 + fdq24 + fdq25 + fdq26 + fdq27. Execute. 27 = All are 'not at all affected' 54 = Average mildly affected 81 = Average, moderatelyaffected 108= Average, severelyaffected 433 fdqsoc FDQ - Social Scores Compute fdqsoc = fdq5 + fdq7 + fdq9 + fdq 16 + fdq 17 + fdq20 + fdq22 + fdq24. Execute . 8= All are 'not at all affected' 16 = Average mildly affected 24 = Average, moderatelyaffected 32 = Average, severelyaffected 434 fdqphy FDQ - Physical Scores Compute fdqphy = fdq2 + fdq4 + fdq8 + fdq 12 + fdq 13 + fdq 19 + fdq27 Execute . . 7= All are 'not at all affected' 14 = Average, mildly affected 21 = Average, moderatelyaffected 28 = Average, severelyaffected NB : PhysicalScoreshasremoved 14 (Driving), 18 (Typing), 23 (Riding a bicycle) and 26 (Sports) from the other categories, as experiencehas shown these generate high numbersof 0 (not applicable)scores. 435 fdqsel FDQ - Self Care Scores Compute fdqsel = fdql 4-fdqlO + fdql5 + fdq2l + fdq25. Execute. 5= 10 = 15 = 20 =

All are 'not at all affected' Average, mildly affected ZP Average, moderately affected Average, severely affected

101

Dystonia -A comprehensiveand longitudinal study in the North East of England

436 fdqlei FDQ - Leisure Scores Computefdqlei = fdq3 + fdq6 + fdq 11. Execute. 3= All are 'not at all affected' 6= Average, mildly affected 9= Average, moderatelyaffected 12 = Average, severelyaffected 437 fdqoth FDQ - Other Physical Scores Computefdqoth=fdq2 + fdq4 + fdqg + fdql2 + fdql3 + fdql4 + fdql8 + fdql9 fdq23 + fdq26 + fdq27. Execute. II= All are 'not at all affected' 22 = Average, mildly affected 33 = Average, moderatelyaffected 44 = Average, severelyaffected NB : Other Physical Scoreshas had 14 (Driving), 18 (Typing), 23 (Riding a bicycle) and 26 (Sports) included along vAth the other categories. This sub-set must not be used in averagingscoresand comparingwith other sub-setaverages. In 'phddata.sav', the 'not applicable' scoresare :fdq 9 14 (Driving) 18 (Typing) 23 (Riding a bicycle) 26 (Sports) 438 disa

Number 150 153 138 91

out of 333 333 333 333

% 45.0% 45.9% 41.4% 27.3%

relatesto Q 27 : Disabling (re day activities) 0= Not at all disabling 10 = Very disabling

439 unco

relatesto Q 27 : Uncomfortable (in social situations) 0= Not at all uncomfortable 10 = Very uncomfortable

440 bcsI

relatesto Graceful vs I= 2= 3= 4= 5= 6= 7=

Awkward

Temp = bcs

Negative, very well described Negative, fairly well described Negative, only slightly described Equally descriptiveor irrelevant Positive, only slightly described Positive, fairly well described Positive, very well described 102

Dystonia -A comprehensiveand longitudinal study in the North East of England

441 442 443 444 445 446 447 448 449 450 451 452 453 454 455 456 457 458 459 460 461

bcs2 bes3 bcs4 bcs5 bcs6 bcs7 bcs8 bcs9 bcslO bcsl I bcsl2 bcs13 bcs14 bcsl5 bcsl6 bcs17 bcsl8 bcsl9 bcs20 bcs2l bcs22

relatesto Lethargic vs relatesto Swift vs relatesto Calm vs relatesto Ugly vs relatesto Rigid vs relatesto Fit vs relatesto Unbalancedvs relatesto Steady vs relatesto Weak vs relatesto Relaxed vs relatesto Masculine vs relatesto Slow vs relatesto Poised vs relatesto Healthy vs relatesto Clumsy vs relatesto Straight vs relatesto Mobile vs relatesto Flawed vs relatesto Uncontrol lable vs relatesto Active vs relatesto Delicate vs;

462

besall

BCS - Total of all scores

Energetic Sluggish Agitated Beautiful Flexible Unfit Balanced Unsteady Strong Tense FemHne (opposite if feminine subject) Fast Unpoised Sick Well-coordinated Twisted Immobile Perfect Controllable Passive Robust

Computebcsall= bcsI+ bcs2 + bcs3 + bcs4 + bcs5+ bcs6 + bcs7 + bcs8 + bcs9 + bcsIO + bcsl I+ bcs12+ bcsl3 + bcsl4 + bcs15+ bcs16+ bcs17+ bcs18 + bcs19 bcs20 + bcs2l + bcs22.Execute. 22 = Extremely negative 88 = Equally distributed 154 = Extremely positive

463 bcsspe BCS - Speed / Strength Scores Computebcsspe= bcs3 + bcs7 + bcsIO + bcsl3 + bcs15+ bcs2l + bcs22. Execute. 7= Extremely negative 28 = Equally distributed 49 = Extremely positive NB : The Body Concept ScaleCoding Frameis shown on page 53 (overleaO

103

Dystonia -A comprehensiveand longitudinal study in the North East of England

BCS Coding Frame

Graceful Lethargic Swift Calm ugly Rigid Fit Unbalanced Steady Weak Relaxed Masculine Slow Poised Healthy Clumsy Straight Mobile Flawed Uncontrollable Active Delicate

1: 2: 7: 6: 1: 2: 1: 2: 7: 6: 7: 6: 1: 2: 7: 6: 1: 2: 7: 6: 1: 2: 1: 2: 7: 6: 1: 2: 1: 2: 7: 6: 1: 2: 1: 2: 7: 6: 7: 6: 1: 2: 7: 6:

3: 4: 5: 4: 3: 4: 3: 4: 5: 4: 5: 4: 3: 4: 5: 4: 3: 4: 5: 4: 3: 4: 3: 4: 5: 4: 3: 4: 3: 4: 5: 4: 3 : 4: 3: 4: 5: 4: 5: 4: 3: 4: 5: 4:

5: 6: 3 : 2: 5: 6: 5: 6: 3: 2: 3: 2: 5: 6: 3: 2: 5: 6: 3: 2: 5: 6: 5: 6: 3: 2: 5: 6: 5: 6: 3: 2: 5: 6: 5: 6: 3: 2: 3: 2: 5: 6: 3: 2:

7 1 7 7 1 1 7 1 7 1 7 7 1 7 7 1 7 7 1 1 7 1

Awkward Energetic Sluggish Agitated Beautiful Flexible Unfit Balanced Unsteady Strong Tense Feminine Fast Unpoised Sick Well-Coordinated Twisted Immobile Perfect Controllable Passive Robust

464 bcspos BCS - Postural / Movement related Compute bespos= bcsl + bcs6 + bcs8 + bcs9 + bcs16+ bcs17 + bcs20. Execute. 7= Extremely negative 28 = Equally distributed 49 = Extremely positive

465 bcseva BCS - Evaluative / Aesthetic Scores Computebcseva= bcs2 + bcs5 + bcsl4 + bcs19. Execute. 4= Extremely negative 16 = Equally distributed 28 = Extremely positive

466 bcsten BCS - Tension scores Computebcsten= bcs4 + bcs11. Execute

.

2= Extremely negative 8= Equally distributed 14 = Extremely positive

104

Dystonia -A comprehensiveand longitudinal study in the North East of England

467 disf

relatesto Disfigurement 0= Not disfigured at all 10 = Extremely disfigured

468 bdil.

relatesto Q 01: Sadness 0= I= 2= 3=

469 470 471 472 473 474 475 476 477 478 479 480 481 482 483 484 485 486 487 488 489

bdi2 bdi3 bdi4 bdi5 bdi6 bdi7 bdi8 bdi9 bdi1O bdill bdi 12 bdi 13 bdi14 bdil5 bdi16 bdil7 bdil8 bdil9 bdi20 bdi21 bdi22

490 bditot

Template= BDI

Not at all condition (described) Mld condition Moderate condition Severecondition

relatesto Q 02: Discouraged relatesto Q 03: Failure relatesto Q 04: Satisfaction relatesto Q 05: Guilty relatesto Q 06: Punished relatesto Q 07: Disappointed relatesto Q 08: Blame relatesto Q 09: Kill relatesto Q 10: Cry relatesto Q 11: Irritated relatesto Q 12: Lost interest relatesto Q 13: Decisions relatesto Q 14: Look relatesto Q 15: Work relatesto Q 16: Sleep relatesto Q 17: Tired relatesto Q 18: Appetite relatesto Q 19: Weight relatesto Q 19: Losing weight by eating less ? relatesto Q 20: Worried relatesto Q 21: Sex

Ternp = Yes No Ternp = BDI

Beck Depression Inventory - Total scores

Computebditot = bdil + bdi2 + bdi3 + bdi4 + bdi5 + bdi6 + bdi7 + bdi8 + bdi9 + bdilO + bdil I+ bdi12 + bdil3 + bdil4 + bdil5 + bdil6 + bdil7 + bdil 8+ bdil9 + bdi2l + bdi22. Execute. 0= No depressionmeasured 66 = Extremely (clinically) depressed

491 sesl

relatesto Q 01 : Satisfied I= 2= 3= 4=

Strongly agree (4) Agree (3) Disagree (2) Strongly disagree (1)

Template = SES

recodedaccording to the coding frame on page 56 (overleaf)

105

Dystonia -A comprehensiveand longitudinal study in the North East of England

492 493 494 495 496 497 498 499 500

ses2 ses3 ses4 ses5 ses6 ses7 ses8 SeS9 seslo

relatesto Q 02: No good relatesto Q 03: Qualities relatesto Q 04: Do things relatesto Q 05: Proud relatesto Q 06: Useless relatesto Q 07: Person of worth relatesto Q 08: Respect relatesto Q 09: Failure relatesto Q 10: Positive attitude SES Coding Frame strongly strongly agree agree disagree disagree 0

1. On the whole, I am satisfiedwith myself 2. At times I think I am no good at all 3. 1 feel that I have a number of good qualities 4. 1 am ableto do things as well as most other people 5. 1 feel I do not have much to be proud of 6. 1 certainly feel uselessat times 7. 1 feel that I am a person of worth, at least on equal 8. 1 wish I could havemore respectfor myself 9. All in all, I am inclined to feel that I am a failure 10. 1 take a positive attitude towards myself 501

4 1 4 4 1 1 4 1 1 4

3 2 3 3 2 2 3 2 2 3

2 3 2 2 3 3 2 3 3 2

1 4 1 1 4 4 1 4 4 1

sestot Self Esteem Scale - Total scores

Compute sestot = sesl + ses2 + ses3 + ses4 + ses5 + ses6 + ses7 + ses8 + ses9 ses10. Execute.

10 = Minimurn score 40 = Maximum score 502

pesteem Positive Self Esteem

Compute pesteem = sesI+ ses3 + ses4 + ses7 + ses10. Execute. 5= Minimum score 20 = Maximum score 503

nesteem Negative Self Esteem

Compute nesteem = ses2 + ses5 + ses6 + ses8 + ses9. Execute. 5= Minimurn score 20 = Maximum score

106

Dystonia -A comprehensiveand longitudinal study in the North East of England

Impact of Dystonia Questionnaire: Q're No. 60 504 date60 Date of Qre 60

dd.mm.yy 8

505 iodl

Template= IOD

relatesto Q 01: Hard time I= 2= 3= 4= 5=

Stronglydisagree Nfildlydisagree Neitheraureeor disagree Mildly agree Stronglyagree

506 iod2

relatesto Q 02: Useless

507 iod3

relatesto Q 03: Miss the things

508 iod4

relatesto Q 04: More dependant

509 iod5

relatesto Q 05: Uncomfortable

510 iod6

relatesto Q 06: Self-sufficient

511 iod7

relatesto Q 02: Inadequate

Template IOD

5= Stronglydisagree(1) 4= Mildly disagree(2) 3= Neitheragreeor disagree(3) 2= Mildly agree(4) 1= Stronglyagree(5) 1

recoded

Template IOD

512 iodtot Impact of Dystonia - Total scores 7= Minimumimpact 14= 25% impact 21 = 50%impact 28 = 75%impact 35 = Maximumimpact Computeiodtot = iodI+ iod2 + iod3 + iod4 + iod5 + iod6 + iod7.Execute. 513 iod8

relatesto Q Page2: AcceptanceStages 1 =Shock 2= Anger 3= Despair 4= Acceptance

107

Dystonia -A comprehensiveand longitudinal study in the North East of England

514 critl

relatesto Questions: Criticism of studY 1

Temp = CRIT.

I= It hashelpedme (hope it hashelpedyou) 2= Difficult without help 3= Expressedmy feelings (emotions) 4= Courtesy/ Respect 5= Consideration 6= Understanding 7= Much appreciated(Thank you) 8= Very professional 9= "No comment" or No criticisms 10 = Too much trouble II= Need more space(to write) 12 = Questionsduplicated 13 = Only'somerelevant (somenot) 14 = Personal/ disquieting 15 = Prefer writing (than talking about it) 16 = Now in remission(can't / difficult to answer) 17 = (Hope it) helpsyoung people 18 = More researchneeded 19 = Inform more GP's (Dr's) 20 = Use simpler words 515 crit2

relatesto Questions:Criticism of study 2

Ternp = CRIT.

516 crit3

relatesto Questions:Criticism of study 3

Temp = CRIT.

Scrittot Total criticisms of study variablecrit I, crit2 and crit3 Multiple ResponseSet - Range I to 20

108

Dystonia -A comprehensiveand longitudinal study in the North East of England

Primary Carer Questionnaire: Q're No. 70 517 date70 Date of Q're 70

dd.mm.yy 8

518 csil

Numeric 4.0

relatesto Q 01: Relationship to person I= Husband 2= Wife 3= Mother 4= Father 5= Son 6= Daughter 7= Carer7not related 8= No primary carer 9= Girl / Boy friend (Live in partner) 10 = Sibling (sister) II= Sibling (brother) 12 = Persondeclined 13 = Aunt

519 csi2

relatesto Q 02: Sleep I= 2= 3= 4=

Template= SES

Strongly agree Agree Disagree Strongly disagree

520 521 522 523 524 525 526 527 528 529 530 531

csi3 csi4 csi5 csi6 csi7 csi8 csi9 csilo csil.I csil2 csil3 csil4

relatesto Q 03: Inconvenient relatesto Q 04: Physical strain relatesto Q 05: Confining relatesto Q 06: Adjust at home relatesto Q 07: Plans Other Q demands 08: to relates relatesto Q 09: Emotional relatesto Q 10: Behaviour relatesto Q 11: Adjustments at work relatesto Q 12: Financial strain relatesto Q 13: Overwhelmed relatesto Q 14: Distressing

532

csitot

Carer's Self Inventory - Total scores

if missing- deduce2 or 3)

13 = Minimum - 'strongly agree' 26'= Average - 'agree' 32 = Average score 33 = Average score 39 = Average - 'disagree' 52 = Maximum - 'strongly disagree'

109

Dystonia -A comprehensiveand longitudi

in the North East of England

Compute csitot = csi2 + csi3 + csi4 + csi5 + csi6 + csi csi 12 + csi 13 + csi 14. Execute. 533

csi15

relates to Q 15: Statement 1

I= Has changedmy life 2= Has not changedlife 3= Patient has changed 4= Affected my emotions 5= Treatment improved it 6= Been very positive 7= Limited socially 8= Had to stop work 9= Been very stressful 10 = He / Sheneedsmy support II= More publicity required 12 = Generalpublic ignorant 13 =I feel useless(unableto help) 14 = Dr's ignorant 15 = More researchneeded 534 csil.6

relatesto Q 15: Statement2

535 csil7

relatesto Q 15: Statement3

$stattot Total of Statements - variable csi15, csi16 and csi17 Multiple ResponseSet - Range I to 15

110

Dystonia.-A comprehensiveand longitudinal study in the North East of England

Environmental Factors Questionnaire: Q're No 80 This Questionnaire is dividedinto five differentsections-1. GeneralQuestions Questions01 to 18- PagesI to 2 2. ChemicalExposure Questions19to 24 - Page 2 3. AllergicReactions Questions25 to 27 - Page 3 4. FoodandDrink Questions28 to 41 - Pages3 to 4 5. OtherEnvironmental Substances Questions42 to 60 - Pages5 to 8 536 date8O Date of Q're 80 537 hand2 relatesto Q 01: Which hand ?

dd.mm.yy 8 Template= Hand

I =Left 2= Right 3= Can't write (illiterate) 538 hand3 relatesto Q 02: Always same hand ? 539 kick

Temp = Yes/No

relatesto Q 03: Kick a ball, which foot ? I =Left 2= Right 3= Ambidextrous

540 thyl

relatesto Q 04: Abnormalities of thyroid gland

541 thy2

relatesto Q 05: Details I= 2= 3= 4= 5= 6=

542 chngl

Temp = Yes/No

Under-active (Myxedema) Over-active Thyroid removed ThyroglossalCyst Nodule on thyroid Enlarged/ swollen

relatesto Q 06: Life pattern

Template = Yes/No

543 chng2 relatesto Q 07: Describe I

= Drug dependency 2 = Bereavement 3 = (Neuro) Surgery 4 = Diabetic 5 = RTA (Car / Cycle) 6 = Divorce 7 = Redundancy 8 = Family Stress III

Dystonia -A comprehensiveand longitudinal study in the North East of England

9= Pregnancy 10 = Working stress(changes) II= Neighbour problems 12 = Industrial injury 13 = SevereNfigraine 14 = Depression 15 = Eyesight impaired 16 = Childhood diseases 17 = Stroke 544 chng3 relatesto Q 07: Year change took place 0= Unknown year 545 546 547 548 549

smokI smok2 smok3 gas soun].

550 soun2

relatesto Q 08: Smoke regularly relatesto Q 09: Ever smoked (regularly) relatesto Q 10: Affected by other's (smoking) relatesto Q 11: Gas appliances relatesto Q 12: Certain sounds

Temp = Yes / No

relatesto Q 13: Describe sound I= 2= 3= 4= 5= 6=

Long banging Screeching/ Shrill Noise SuddenNoise Buzz, Hiss, etc (Vibrating, tapping, strong winds) Loud Noise / Music Have Tinnitus

551 sunI

relatesto Q 14: Bright light / sunlight

552 sun2

relatesto Q 15: Describe how

Temp = Yes / No

I= (Causes)Blinking 2= Eyes shut (screw up) 3= Headaches/ migraines(pain behindeyes) 4= Blurred vision 5= Photosensitive 6= Makes spasmsworse 7= Dizzy (walking in bright light) 8= Warmth helps spasms 9= Allergic reaction (to sunlight) 10 = Eyes water 553 watl

relatesto Q 16: Drinking water I= Spring 2= Well 3= Mains 4= Filtered 5= Bottled 112

Dystonia -A comprehensiveand longitudinal study in the North East of England

554 wat2

relatesto Q 17:Mains water I =Fluoridated 2= Soft 3= Hard 4= Softened 5= Don't know

555 spma

relatesto Q 18a:Stress

Template= spm

I= Better 2= Unchanged 3= Worse 556 557 558 559 560 561 562 563 564 565 566 567 568 569 570 571 572 573 574 575 576 577

spmb spmc spmd spme spmf spmg spmh spmi spmj spmk SPH spmm spmn spmo spmp spmq spmr spms spmt spmu spmv spmw

relatesto Q l8b: Relaxation relatesto Q 18c:Fatigue relatesto Q 18d:Emotion relatesto Q l8e: Distraction relatesto Q 18f Self-Consciousness relatesto Q 18g:SocialSituations relatesto Q 18h:Heat relatesto Q 18i: Cold relatesto Q 18j:Walking relatesto Q 18k:Running relatesto Q 181:Carrying objects relatesto Q 18m:Writing relatesto Q l8n: Sleep relatesto Q l8o: Lying on back relatesto Q 18p:Lying on side relatesto Q 18q:Pre-MenstrualCycle relatesto Q l8r: During Menstrual Cycles relatesto Q 18s:BetweenMenstrual Cycle relatesto Q 18t: On wakening relatesto Q 18u:During morning relatesto Q 18v:During afternoon relatesto Q 18w:During evening

578 579 580 581 582 583

cheml chem2 chem3 chem4 chem5 chem6

relatesto Q 19: Exposed to chemicals relatesto Q 20: Crop spraying relatesto Q 21: Industrial pollution relatesto Q 22: Industrial accident relatesto Q 23: Chemical fire relatesto Q 24: Other type

Temp = Yes / No

113

Dystonia -A comprehensiveand longitudinal study in the North East of England

584 chem7 relatesto Q 24: Other types of chemicalsexposedto = Photocopying 2= Treated grass 3= Fibre glassresin 4= Woodworm / dry rot (timber treatment) 5= Mercury 6= ScienceLab (worked in) 7= Chlorine 8= Shipyard Chernicals 9= Asbestos 10 = Major Tranquillisers II= Paint (worked in factory or worked with) 12 = Pharmacy(worked in) 13 = ChemicalFactory (worked in) 14 = Traffic pollution (garageforecourt) 15 = Embalmers(worked in) 16 = Building Chemicals(DPC, Sealocrete,etc) 17 = Military Service 18 = Concrete CleaningFluids 19 = Military Warfare (ChemicalWeapons) 20 = Nuclear power 21 = U/G Explosives 22 = SteelMills (worked in) 585 alg

relatesto Q 25: Allergic problems Syntaxfile: 'phd-env.sps'-= 14 calculations

Temp = Yes / No

IF (alg EQ 2) alga= 2. IF (alg EQ 2) algb = 2. [etc, continuesuntil M (alg EQ 2) aign = 2. Execute. This formula only used if no allergic problemsat all havebeennotified. 586 587 588 589 590 591 592 593 594 595 596 597 598 599

alga algb algc algd alge algf algg algh algi algi algk algI algm algn

relatesto Q 26a: Asthma relatesto Q 26b: Migraines relatesto Q 26c: Persistent fatigue relatesto Q 26d: Hay Fever relatesto Q 26e: Arthritis relatesto Q 26f- Hyperactivity relatesto Q 26g: Rhinitis relatesto Q 26h: Mouth Ulcers relatesto Q 26i: Stomach Ulcers relatesto Q 26j: Wind / Bloating relatesto Q 26k: Eczema relatesto Q 261:Puffy ankles / hands face relatesto Q 26m: Uticaria / Hives relatesto Q 26n: Diarrhoea

114

Dystonia -A comprehensiveand longitudinal stud),in the North East of England

600 algo

relatesto Q 26m: Allergic to ... I= Metal (incl Zinc) 2= Drugs (incl.Penicillin, Clonazepam,etc) 3= Skin Allergies (incl Dennatitis, Psoriasis,etc) 4= Not an allergy (incl Heart Bum, Constipation) 5= Eggs (severe)

601 algp

relatesto Q 27: Blood relatives

Temp = Yes / No

602 fadl 603 fad2 604 facla

relatesto Q 28: Food and Drink relatesto Q 29: Spasms worse relatesto Q 30a: Pork

Temp = Yes No Temp = Yes No Temp = fad

0= I= 2= 3= 605 606 607 608 609

fadb fadc fadd fade U3

Not affected at all Mildly affected Moderately affected Severelyaffected

relatesto relatesto relatesto relatesto relatesto

Q 30b: Beef Q 30c: Red meat Q 30d: White meat Q 30c: Fish or Shellfish 1 Q 30e: Fish or Shellfish 2

I =Fish 2= Shellfish 3 =Both 610 611 612 613 614 615

fadf fadg fadh fadi fadj M4

relatesto relatesto relatesto relatesto relatesto relatesto

Q 30f Wheat Q 30g: Rice Q 30h: Corn Q 30i: Oats Q 30j: Other Cereals 1 Q 30j; Other Cereals 2

Temp = fad

I= (Brazil) Nuts 2= Pastry 3= (too much) Fat 4=3 types of Wheat 616 617 618 619 620

fadk fadl fadm fadn fado

relatesto Q 30k: Milk relatesto Q 301:Egg relatesto Q 30m: Cheese relatesto Q 30n: Yoghurt relatesto Q 30o: Other Dairy Products I

Temp = fad

115

Dystonia -A comprehensiveand longitudinal study in the North East of England

621 fad5

622 623 624 625

fadp fadq fadr fad6

relatesto Q 30o: Other Dairy Products 2 1= Cottage cheese 2= (fresh) Cream Coffee Q 30p: to relates relatesto Q 30q: Tea relatesto Q 30r: Other liquids 1 relatesto Q 30r: Other liquids 2 I= 2= 3= 4= 5= 6= 7= 8=

626 fads 627 fadt 628 fadu 629 fad7

Alcohol - undefined Alcohol - Gin Alcohol - Wine OrangeJuice Low C Choc & Vinegar Tap Water Soft drinks (cans) Fruit drinks & Vinegar

relates to relates to relates to relates to

Q Q Q Q

30s: Citrus fruit 30t: Potato 30u: Other Fruit & Veg 1 30u: Other Fruit & Veg 2

I= Strawberries 2= Carrots & Tomatoes 3= Parsnip 4 =.Banana 5= Oranges 6= 'Some' vegetables? 630 631 632 633

fadv fadx fady fadz

relatesto relatesto relatesto relatesto 0= I= 2= 3=

634 635

Q 30v: Yeast Q 30x: Chocolate Q 30y: Sugar Q 30z: Other Foods

Not affected at all Mildly affectedby curries Moderately affectedby curries Severelyaffectedby red wine

excl

relates to Q31: Exclusion diet

Temp = Yes No

cravI

(foods) Crave Q 32: to relates

Temp = Yes No

116

Dystonia -A comprehensiveand longitudinal study in the North East of England

636 crav2

relatesto Q 33: Foods craved (which) I= Chips & Fruit cake 2= Choc & Alcohol 3= Ribena 4= Alcohol 5= Tea & Coffee 6= Chocolate 7= Tea, Choc & Cream 8= Bananas& Crisps 9= Choc & Butter 10 = Choc & Sweet Food II= Anything Sweet 12 = Lucozade - Dry mouth 13 = Hot - Spicy Foods 14 = High Fat Foods (Chips, Cheese,etc) 15 = Garlic 16 = Fruit Juice (OrangeJuice) 17 = Tea, NElk Pudding (Ice Cream) 18 = Coffee 19 = Choc and Yoghurt (Drinks) 20 = Horlicks 21 = Choc and Coffee

637 tea 638 coff 639 decal

relatesto Q 34: No of cups of tea (in a day) relatesto Q 35: No of cups of coffee (in a day) relatesto Q 36: Decaffeinated I =Yes 2 =No 3= Don't drink coffee

640 deca2 relatesto Q 37: Describe how ? I= Undec makesspasmsworse 2= Dee makesspasmsbetter

641 winel

relatesto Q 38: Wines I =Yes 2=No 3= Don't drink alcohol

117

Dystonia,-A comprehensiveand longitudinal study in the North East of England

642 wine2

relatesto Q 39: Describe how ? I= Have more confidence 2= Relaxesspasms 3= Severelyaffected (adversely) 4= Alcoholic (recovering) 5= Nfildly affected (adversely) 6= Slightly sick 7= Dries up mouth 8= Go to sleep 9= Slight headache 10 = Loosensbowels (Colitis) II= Reactswith drugs 0

643 colal

relatesto Q 40: Colas I =Yes 2 =No 3= Don't drink them

644 cola2

relatesto Q 41: Describe how ? I= Choke when drinking 2= Slight hiccups 3= Feel edgy / nervous 4= Tongue - sensitive/ sore 5= Dries up mouth 6= Aids digestion 7= Spasmsin stomach 8= Wind and bloating 9= Upset stomach(nausea) 10 = Worse spasms(next day)

645 envI

Environmental factors (method of entering data only) Syntaxfile : 'phd-env.sps'= 79 calculations I= No data for rest of series

IF (env EQ 1) envaa= 0. IF (enc EQ 1) envab= 0. [etc, continuesuntil IF (env EQ 1) envdz= 0. Execute. 646 647 648 649 650

envaa envab envac envad envae

relatesto Q 42aa: New mown grass relatesto Q 42ab: Trees relatesto Q 42ac: Long grass relatesto Q 42ad: Bay relatesto Q 42ae: Other pollens

651 652 653 654

envaf envag envah envai

relatesto Q 43af. Damp humid days relatesto Q 43ag: Old houses relatesto Q 43ah: Fungus relatesto Q 43ai: Moulds

Template= fad

118

Dystonia -A comprehensiveand longitudinal study in the North East of England

655 656 657 658 659 660 661

envaj envak enval envam envan envao envap

662 663 664 665

envaq relatesto envar relatesto envas relatesto envat relatesto

666 667 668 669 670 671

envau envav envaw envax envay envaz

relatesto relatesto relatesto relatesto relatesto relatesto

672 673 674 675 676 677 678

envba envbb envbc envbd envbe envbf envbg

relatesto Q 47ba: Degreasers relatesto Q 47bb: Deodorants relatesto Q 47bc: Hairsprays relatesto Q 47bd: Polishes relatesto Q 47be: Insecticides relatesto Q 47bf. Pesticides relatesto Q 47bg: Air Freshener

679 680 681 682 683 684 685

envbh envbi envbj envbk envbl envbm envbn

relatesto Q 48bh: After-shave relatesto Q 48bi: Creams relatesto Q 48bj: Deodorants relatesto Q 48bk: Perfume relatesto Q 48bl: Powder relatesto Q 48bm: Make-up relatesto Q 48bn: Shampoo

686 687 688 689 690

envbo envbp envbq envbr envbs

relatesto Q 49bo: New carpets relatesto Q 49bp: Linoleum relatesto Q 49bq: Floor Tiles relatesto Q 49br: Sealer relatesto Q 49bs: Adhesive

691 692 693 693

envbt envbu envbv envbw

relatesto relatesto relatesto relatesto

relatesto Q 43aj: Dust relatesto Q 44ak: Dogs relatesto Q 44al: Cats relatesto Q 44am: Horses relatesto Q 44an: Rodents relatesto Q 44ao: Birds relatesto Q 44ap: Feathers

Template= fad

Q 45aq: Bee stings Q 45ar: Wasp stings Q 45as: House dust Q 45at: Mites Q 46au: Odour of pines Q 46av: Pine products Q 46aw: Houseplants Q 46ax: Manure Q 46ay: Silage Q 46az: Rotting vegetation

Q 50bt: Carpet backing Q 50bu: Cushion Q 50bv: Upholstery Q 50bw: Padding

119

Dystonia -A comprehensiveand longitudinal study in the North East of England

695 696 697 698 699 700 701

envbx envby envbz envca envcb envcd envcc

Template= fad relatesto Q5 Ibx: Ammonia relatesto Q 51by: Bleaches relatesto Q 51bz: Detergents relatesto Q5 Ica: Liquid polishers relatesto Q51 cb: Silver polish relatesto Q51 cd: Furniture polish (Note - out of sequence) relatesto Q51 cc: Carpet shampoo (Note - out of sequence)

702 703 704 705 706 707 708 709

enved envce envcf envcg envch envci envqj envck

relatesto Q 52cd: Heating Oil (Note - add referencenumber) relatesto Q 52ce: Gas relatesto Q 52cf.- Paraffin relatesto Q 52cg: Calor / Butane relatesto Q 52ch: Coal Fire relatesto Q 52ci: Charcoal relatesto Q 52cj: Burning Tar relatesto Q 52ck: Burning Rubber

710 711 712 713 714

envcI envem envcn envco envcp

relatesto Q 53cl: Petrol fumes relatesto Q 53cm: Oil relatesto Q 53cn: Diesel fumes relatesto Q 53co: Car Upholstery relatesto Q 53cp: Exhaust fumes

715 716 717 718 719 720

envcq enver envcs envct envcu envcv

relatesto Q 54cq: New paint relatesto Q 54cr: Paint stripper relatesto Q 54cs: Turpentine relatesto Q 54ct: Varnish relatesto Q 54cu: Oils relatesto Q 54cv: Fixative

721 722 723 724

envcw envcx . envcy envcz

relatesto relatesto relatesto relatesto

Q 55cw: Cigarettes Q 55cx: Cigars Q 55cy: Pipe Q 55cz: Cigarette smoke

725 726 727 728 729 730 731 732 733 734 735 736 737

envda. envdb envdc envdd envde envdf envdg envdh envdi envdj envdk envdl envdm

relatesto relatesto relatesto relatesto relatesto relatesto relatesto relatesto relatesto relatesto relatesto relatesto relatesto

Q 56da: Lighter fuel Q 56db: Carbon Tetrachloride Q 56dc: Glues Q 56dd: Newsprint Q 56de: White Spirit Q 56df Dry Cleaning fluid Q 5.6dg:Methylated Spirits Q 56dh: Surgical Spirit Q 57di: Chlorine Q 57dj: Bitumen Tar Q 57dk: Asphalt Q 57dl: Weedkillers Q 57dm: Mothballs

120

Dystonia -A comprehensiveand longitudinal study in the North East of England

738 739 740 741 742 743 744 745 746 747 748 749 750 751

envdn envdo envdp envdq envdr envds envdt envdu envdv

relatesto Q 57dn: Disinfectants Pools Swimming Q 57do: to relates Additives Q Food 57dp: to relates in Food Residues Pesticide Q 57dq: to relates in Water Q 57dr: Pollutants to relates fruit Waxes Q 57ds: to and veg on relates relatesto Q 57dt: Tartrazine in Resin Phenolic Q 57du: cans to relates relatesto Q 57dv: Formaldehyde

envdw envdx envdy envdz env2

relatesto Q 58dw: Plastics Coated Q 58dx: to paper relates fabric New Q 58dy: to relates Other 1 Q 58dz: to relates What Q 58dz: to other smells relates I= 2= 3= 4= 5= 6= 7= 8=

Template= fad

Rubbishbins Carrots Burning smells Fishy smells Wood smoke 'Creosote' Onions Rubefacientcream

752 envea relatesto Q 59dz: Other 2 753 env3 relatesto Q 59dz: What others 2?

Temp = fad

I= Bath cleaner 2= (some) Lagers 3= Body odour 4= Food colouring 5 ='Penicillin' 6 ='Maxolon' 7= 'Aspirin' 8= EssentialOils 9= Drug (reaction) 10 = Eels II= 'Scotchguard' 12 = 'Lanolin' 13 = 'Algipan' Spray 14 = (Garden) Soil 15 = Straw dust 754 enveb relatesto Q 59dz: Other 3

Template= fad

121

Dystonia -A comprehensiveand longitudinal study in the North East of England

755 env4

relatesto Q 59dz: What others 3? I= Unwashedbodies 2= Tomatoes 3= 4= 5= 6=

Flowers Aspirin Rats Hair Dye

756 comml. relatesto Q 60: Comment I I= Worse, activity/walking : Better resting/relaxed 2= Worse, self-conscious 3= Better, busy 4= Better, no stress : Worse with stress 5= Worse, reading / TV 6= Worse, worry 7= Better, sun / warmth : Worse, cold / damp 8= Currently in remission 9= Counselling helps 10 = Worse, miss inj. : Better with inj / medication II= High Voltage Electricity: o/h lines ?? 12 = Worse, tired : Better, sleep / in mornings 13 = Helped by surgery 14 = Worse, over time 15 = Worse, bright (sun)light 16 = Worse, Depression / Anxiety 17 = Worse, (severe) climatic changes 18 = Worse lie on affected side: Posture affects spasms 19 = Poss. link with drugs / solvents 20 = Worse (on) windy days 21 = "No comment / Don't know" 22 = Better (with) positive thoughts (or laughter) 23 = Unaffected (by) anything 24 = Definite pattern (but) why ? 25 = Better (with) alcohol 26 = No definite pattern 27 = Suspect hormonal fink 28 = Worse, before period : Better, during period

757 comni2 relatesto Q 60: Comment 2 758 conun3 relatesto Q 60: Comment 3 ScommtotTotal - Comments - variable comml, comm2 and comm3 Multiple ResponseSet - Range I to 28

122

Dystonia -A comprehensiveand longitudinal study in the North East of England

DiagnosisQuestionnaire: Q're No 81 759 date8l Date of Q're 81 or date of death 760

dd.mm.yy 8

surv8l Participation in Q're 81 I= participated 2 = not returned 3 = deceased 4 = not issued 5 = Info from interview

761 sped

relatesto Question I: Specialist 1

Template = Specialist

I= OrthopaedicSurgeon 2= Rheumatologist 3= Psychiatrist 4= Neurologist 5= ENT Surgeon 6= Physiotherapist 7= Ophthalmologist Surgeon 8= Neuro Surgeon 9= Aromatherapist 10 = Osteopath II= Meditation Spec. 12 = Psychologist 13 = Chiropractor 14 = SpeechTherapist 15 = Plastic Surgeon 16 = Chiropodist 17 = Gynaecologist 18 = Haematologist 19 = Paediatrician 20 = PaediatricNeuro. 21 = Pain Spec. 22 = Orthodontic Surgeon 23 = Unknown Consultant 24 = Geriatrician 25 = Immunologist 762 spec2 relatesto Question I Specialist 2 763 spec3 relatesto Question 1 Specialist 3 764 spec4 relatesto Question I Specialist 4 $spectotTotal - Specialists - variable specl, spec2,spec3and spec4 Multiple ResponseSet - Range I to 25

123

Dystonia -A comprehensiveand longitudinal study in the North East of England

765 altl

relatesto Question2: Alternative treatment 1 Temp = Alternative I= Osteopathy 2= Acupuncture 3= Hypnosis/ Hypnotherapy 4= Physiotherapy 5= Psychotherapy 6= SpeechTherapy 7= Homeopathy 8= Counselling 9= Experimentaldrugs (GuineaPig) CD 10 = Traction II =Yoga 12 = Chiropractic 13 = Surgery 14 = Behavioural.Therapy 15 = Reflexology 16 = Hydrotherapy 17 = Aromatherapy 18 = Faith Healing (Healing Hands) 19 = Relaxationtechniques 20 = Heat treatment/ Elec shock 21 = Manipulation (under anaesthetic)

766 alt2 767 alt3 768 alt4

relatesto Question2 Alternative treatment 2 relatesto Question2 Alternative treatment 3 relatesto Question2 Alternative treatment 4

$alttot Total - Alternative treatments - variable alt I, alt2, alt3 and alt4 Multiple ResponseSet - Range I to 21 0 769 diff3

770

diff4

relatesto Question3: Time to obtain a diagnosis Comparedto diff betweenonset and diagnosis Compare diffl

& diff3 (in years)

Compute diff4 = diffl - diff3 Execute. .

124

Dystonia -A comprehensiveand longitudinal study in the North East of England

Dystonia Nurse Practitioner Questionnaires : Q'res No. 91 to 94 771 stat3

dnp status

Numeric 4.0

I= Home -completed 2= Home - incomplete 3 =Home -declined 4= Home - deceased 5= Clinic - completed 6= Clinic - incomplete 7= Clinic - declined 8= Clinic - deceased 9= Not in study 772 dnp

dnp location

String 4

C= Clinic H= Home

N= Not in dnpstudy 773 dnpno dnp project number

String 5

COI to C66 inclusive HO I to H64 inclusive

774 date9l Date of Qre 91 775

age9l

relates to Age (of patient) as of date9l

dd.mm.yy 8 Numeric 4.1

Computeage9l = YRMODA (xdate.year(date9l),xdate.month(date9l), xdate.mday(date9l)) / 365.25 - YRMODA (xdate.year(dob), xdate.month(dob),xdate.mday(dob))/365.25. Execute. Compute age9l = (((age9l * 10) + 0.5) - (MOD(((age9l Execute.

* 10) + 0.5), 1)))/l 0.

776 dnplOl No of injections ? (since first started) 777 dnp102 No of injectors ? (since first started) 778

dnp103 How much time ? (in minutes)

779

dnp104 How often hurt ? (number of times)

0= Never hurt 30 = Hurt everytime

125

Dystonia -A comprehensiveand longitudinal study in the North East of England

780 dnp105 Best Knowledge I I= 2= 3= 4= 5=

Very good Good Average Poor Very Poor

Temp = dnp-rating ) ) ) ) )

Subjectiveranking by the respondent in answeringthe questionsrelating to the injectors

781 dnp106 Worst Knowledge 1 782 dnp107 Best Attitude 1 783 dnp108 Worst Attitude 1 784 dnp109 HMH Service now I

Temp = dnp-service

I= Seenpromptly (usually) 2= Staff efficient 3= No complaints 4= Good - Very good (excellent) 5= Occasionalwaiting 6= ReceptionistVG. 7= Friendly atmosphere(tea / coffee) 8= Consultation(too) short 9= Production Line (Cattle Market) 10 = Better before (ie reception moved generally) II= Occasionalirj. hurt 12 = Screen(no privacy) 13 = Car parking (difficult) 14 = Ambulance(travel time) 15 = Canteen(no signs/ no drop in facilities) 16 = Different injectors 17 = Not (well) organised 18 = Explain clearly (needed) 19 = HMH better than other (hospitals) 20 = Better with sameDr. 785

dnpIlO

HMHServicenow2

Temp = dnp-service

$hmhtot Total - HMH Service now - variable dnp109 and dnpI 10 Multiple ResponseSet - Range I to 20 786 dnpl II Type of spasms ? 787 dnpl 12 What is the cause ? 788

dnpl 13 Spasms worse 1

I= 2= 3= 4=

Temp = dnp-rating

Temp = dnp-worse

No, nothing Smoking Social Situations Stress/ Anxiety 126

Dystonia -A comprehensiveand longitudinal study in the North East of England

5= Angry / Upset / Tense 6= Cold 7= Physicalactivity 8= Tiredness 9= Worry 10 = BT cycle (too long) II= Sneezing 12 = Reading/ (Watching) TV 13 = Bright lights 14 = Wind 15 = NEgraines 16"=Agoraphobia 17 = Loud Noise 18 = Depression 19 = Caffeine 789 dnp114 Spasmsworse2 $wortot Total - Spasmsworse - variable dnp113 and dnp114 Multiple ResponseSet - Range I to 19 790 dnp115 Spasmsbetter I

Temp = dnp-better

I= Operation 2= Medication 3= Sleep 4= Heat (Warmth) 5= Bot. Tox.

6= Rest (Relaxation) 7= In Remission 8= Smoking (Cannabis) 9= Positive Thoughts (Happy disposition) 10 = Nothing (except BT) II= Alcohol 12 = Chewing gum 13 = Good days/ bad days 14 = TENS Machine 15 = GesteAntagoniste 791

dnp 116 Spasms better 2

$betttot Total - Spasms better - variable dnpl 15 and dnpI 16 Multiple ResponseSet - Range I to 15

127

Dy'stonia-A comprehensiveand longitudinal study in the North East of England

792

date92 Date of Q're 92

793 age92 Age as of date92

dd.mm.yy 8 Numeric 4.1

Compute age92= YRMODA (xdate.year(date92),xdate.month(date92), xdate.mday(date92))/ 365.25 - YRMODA (xdate.year(dob), xdate.month(dob),xdate.mday(dob))/365.25. Execute. Compute age92= (((age92 * 10) + 0.5) - (MOD(((age92 * 10) + 0.5), 1)))/l 0. Execute. 794 dnp201 x injected last qre ? 795 dnp202 x injectors last q're ? 796 dnp203 How much time ? (in minutes) 797 dnp204 x injections hurt ? 0= Never hurt 798 799 800 801

dnp205 dnp206 dnp207 dnp208

Best Knowledge 2 Worst Knowledge 2 Best Attitude 2 Worst Attitude 2

802 dnp209 Service improved I

Temp = dnp-rating

Temp = dnp-improved

I= Can't be improved 2= More flexible (appointmentsdate and time) 3 =No, nothing 4= Changeinj. cycle (too long) 5= Good - Very good 6= Very polite / efficient 7= Bot Tox (bad batches) 8= Annual Review (for eachpatient) 9= Time with the doctor 10 = No comment/ complaints II= Samedoctor (eachtime) 12 = Environment (of the clinic) 13 = Screen(more privacy) 14 = It has improved 15 = Time waiting (-ve) 16 = Waste of toxin (Availability) 17 = Doctor's attitude 18 = DSS Expenses(paid at clinic) 19 = Increaseno of DNP's (or Dr's) 20 = (Wish I was / Glad I am) on Home List 21 = Car Parking 22 = Waiting - too long 23 = Short staffed (occasionally

128

Dystonia -A comprehensiveand longitudinal study in the North East of England

803 dnp210 Service improved 2 Simptot Total - Service improved - variable dnp209 and dnp210 Multiple ResponseSet - Range I to 23 804 805 806 807

dnp2l I dnp2l2 dnp2l3 dnp2l4

Aetiology of dystonia ? Cause or primary (dystonia) ? Cause of secondary (dystonia) ? Difference between Ist and 2nd ?

Temp = dnp-rating

808 date93 Date of Q're 93

dd.mm.yy 8

809

Numeric 4.1

age93

Age as of date93

ComPuteage93= YRMODA (xdate.year(date93),xdate.month(date93), xdate.mday(date93))/ 365.25 - YRMODA (xdate.year(dob), xdate.month(dob),xdate.mday(dob))/365.25. Execute. ComPute age93 = (((age93 * 10) + 0.5) - (MOD(((age93 * 10) + 0.5), 1)))/l 0. Execute.

810 dnp301 x injected last q're ? 811 dnp302 x injectors last qre ? 812 dnp303 How much time ? (in minutes) 813 dnp304 x injections hurt ? 0= Never hurt 814 dnp305 Best Knowledge 3 815 dnp306 Worst Knowledge 3

Temp = dnp-rating

816 dnp307 Best Attitude 3 817 dnp308 Worst Attitude 3

818 dnp309 Service at end I

Temp = dnp-end

I= No, nothing (no comment) 2= Very satisfied(happy) 3= Good - Very good (service) 4= Secondto none/ Excellent 5= Changeddays (Wed to Tue) 6= Changedtime (am to pm) 7= Train Di's (talk to patients) 8= Continue/ Finance = Self-conscious(open plan) _9 10 = Counsellingservice II= Screen(no longer up) 12 = Talk to Dr (time)

129

Dystonia -A comprehensiveand longitudinal study in the North East of England

13 = Waiting occasionallylong 14 = Cancelledappointments 15 = Delayed appointments 16 = Improved attitudes 17 = Improved time (Dr) 18 = Savestime / money 19 = Clinic busier now 819 dnp3lO Serviceatend2 $servtot Total - Service from HMH variablednp309 and dnp310 Multiple ResponseSet - Range I to 19 820 dnp311 Idiopathic dystonia ? 821 dnp312 Basal ganglia ?

Temp = dnp-rating

822 dnp3l3 Treatment improved? I =Yes 2=No 3= About the same

823 dnp3l4 Service Improved ? (comparedto one year ago) I =Better 2= About the same 3= Worse 824 dnp401 Whole project I

Temp = dnp-project

I= Less stressat home (more relaxed) 2= DNP is the best (Dr Barnes/ cant be bettered) 3= Better now diagnosed(happiernow settled) 4= Good idea (scheme/ brilliant) 5= You are listenedto 6= DNP effective (Condition explained) 7= Good - very good 8= Savetravel time / savesmoney 9= Dr's time (limited) 10 =I have control II= Sameperson/ DNP personal(relationship) 12 = DNP for easycases(long distance) 13 = HMH for hard cases(short distance) 14 = Fall back (ie BW 15 = Wish (I had been) on home list 16 = Annual (fMffD review 17 = Staff - good at clinic 18 = Clinic now busier 19 = (DNP takes) pressureoff clinic 130

Dystonia

in North East England longitudinal the study of comprehensive and -A

825 dnp402 Whole project 2 826 dnp403 Whole project 3 Swholtot Total Whole project variable dnp40l, dnp402 and dnp403 Multiple ResponseSet Range I to 19 827 dnp404 Home or clinic? I= Home 2= Clinic 3= Either 828 dnp405 Why home 1?

Temp = dnp-home

I= More relaxed (secure) 2= 3= 4= 5= 6= 7= 8= 9= 10 = II= 12 = 829

More convenient (time / travel / money) More time (to discuss problems) Same person (continuity of treatment) DNP listens (trust) DNP explains (condition / treatment) Personal attention (of DNP) Self conscious (in clinic / on bus) Difficulty walking (travelling) Time (off work) Husband / Patient / My No reason given Save money / time / work (NHS Ambulance)

dnp406 Why home 2

$hometot Total - Why home variable dnp405 and dnp406 Multiple ResponseSet - Range I to 12 830 dnp407 Why clinic 1 I= 2= 3= 4= 5= 6= 7= 8= 9 10 831

Temp = dnp-clinic

Dr's advice (neededsometimes) Seeother people (lessisolated) Fixed time (better for me) Day out (able to do other things / shopping.etc) Fits (in) with work (times) Happy for either method No travel problems Family unawareof problems Others needDNP more than me Coffee / tea (Free of charge/ facilities good)

dnp408 Why clinic 2

$clintot Total - Why clinic - variable dnp407 and dnp408 Multiple ResponseSet - Range I to 10 131

Dystonia -A comprehensiveand longitudinal study in the North East of England

832 dnp409 Other comments I

TemP= dnp-comments

I= Results(published) 2= Very satisfied(happy) 3= Clinic (became)too busy 4= Personalrelationship (with DNP) 5= Your family also 6= Many thanks 7= Awareness(amongst G.P's) 8= Medical Prize (students) 9= Organisedwell 10 = Clinic patients(some resentful) II= More explanation(at clinic needed) 12 = CounseflingService (VG. ) 13 = Clinic improved (over past year) 14 = No comment 15 = Offer research(for future) 16 = Hope it continues(want it to) 17 = Ambulancetravel (long time) 18 = No differencein treatment (home/ clinic) 19 = Home - more personal 20 = HMH better than NRI (other hospitals) 21 = NRI - long waiting 22 = NRI - mixed clinic 23 = Longer inj. cycle (now) 24 = BT strength (batchesnot standard) 25 = More DNP's (needed) 26 = appointmentscancelled- why ? 833 dnp4l 0 Other comments 2 834 dnp4ll Other comments 3

$othtot Total - Other comments - variable dnp409, dnp4lO and dnp4lO Multiple ResponseSet - Range I to 26 835 diff7

DNP Duration (of study)

Compute diff7 = age93- age9l Execute. . Compute diff7 = (((diff7 * 10) + 0.5) - (MOD(((diff7 * 10) + 0.5), 1)))/10. Execute.

132

Dystonia -A comprehensiveand longitudinal study in the North East of England

836 date98 End of survey or death

dd.mm.yy8

End of survey =01.0 1.99, being 5.56 yearsfrom the start 837 age98 Age at end or death

Numeric 4.1

Computeagge98 = YRMODA (xdate.year(date98),xdate.month(date98), xdate.mday(date98))/ 365.25 - YRMODA (xdate.year(dob), xdate.month(dob),xdate.mday(dob))/365.25. Execute. Computeage98= (((age98 * 10) + 0.5) - (MOD(((age98 * 10) + 0.5), l)))/10. Execute. 838 time3

Time in study (years) ie time betweenbeginning& end.

Numeric 4.1

Computetime3 = YRMODA (xdate.year(date98),xdate.month(date98), (xdate. / YRMODA 365.25 year(datelO), xdate.mday(date98)) xdate.month(dateI 0),xd ate.mday(date10))/ 365.25 . Execute. Compute time3 = (((time3 * 10) + 0.5) - (MOD(((time3 * 10) + 0.5), 1)))/10. Execute.

839 recodel Time in study Recodedtime 3 into categoriesasbelow :I= Over 5.50 years 2=5.00 - 5.49 years 3=4.50 - 4.99 years 4=4.00 - 4.49 years 5=3.50 - 3.99 years 6=3.00 - 3.49 years 7=2.50 - 2.99 years 8=2.00 - 2.49 years 9=1.50 - 1.99 years 10 = 1.00 - 1.49 years II=0.50 - 0.99 years 12 = 0.00 - 0.49 years 13 = Negative years

133

Dystonia -A comprehensiveand longitudinal study in the North East of England

840 wbsl

Welfare Benefits Service I= 2= 3= 4= 5= 6= 7= 8= 9=

841 wbs2

Referredto Welfare Benefits Adviser Advised by WBA on telephone Advised by WBA in person CompletedBenefit Application Successfulapplication Applied for review / appeal Succesfulreview / appeal Unsuccessfulreview / appeal Unknown result

WBS - Referral Date 9804 = April 1998 - start of WBS during ESD 9812 = December 1998 - end of WBS in ESD 9901 = January 1999 - continuation of WBS

842 wbs3

WBS - Referral (by whom) DM David (or Connie) Medd GB Ginger Butler HC Harry Crow JW John Whitaker ME Maurice Hawthorne PW Peter Williams

134

Dystonia -A comprehensiveand longitudinal study in the North East of England

Templates Lists EuroQol and SF 36 Questionnaire No 10 Order

Alpha

Name of Template

Columnsusing this template

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17

46 55 1 16 5 37 35 13 63 67 32 38 43 39 62 49 53

MOBI]LITY SELF-CARE ACTIVITY DISCOMFORT ANXIETY HEALTH GEN. HEALTH CONTARED VIGOROUS YES-NO EXTENT HOW LIFE ELL TYPE PARTNER QUALITATIVE

16,89. 17,90. 18,91. 19,92. 20,93. 28,101. 29,102. 30,103. 31-40,104-113 42-45,46-48,115-118,120-122 51,53,124,126. 52,125. 55-62,64,66,128-135,137,139 68-71,141-144. 73,146. 76-83,149-156. 84,157.

Demographic Questionnaire No 20 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33

30 54 8 28 17 34 65 45 64 51 57 42 12 40, 9 3

EMPLOY SEG BENEFITS DOCTOR DISTRIBUTION FOCAL WHO MEDICATION VvTIEN PREVIOUS SIDE-EFFECTS LAST COMORBIDITY INJECTIONS BODYI AIM

175,176. 177-181. 184-186. 197,198. 204,219,221,223. 205-208. 218,220,222. 224,227,230,232,234. 226,229. 237-241. 242,243,259,260. 244,261. 245-248. 250-255. 257,258. 277,278.

135

Dystonia -A comprehensiveand longitudinal study in the North East of England

PSR Questionnaires : Nos. 30 to 70 Order

Alpha

Name of Template

Columnsusing this template

34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52

68 31 66 29 50 52 61 47 14 10 44 48 2 6 7 56 41 15 60

YES-NO EVENT YEAR EFFECT PHYSICAL PSYCHIATRIC TREATMENT MOVEMENTS CONTROL BODY2 LONG PARTIAL AFFECTED (FDQ) BCS BDI SES IOD CRITICISM STATEMENT

286,289,290,322,331,338,339, etc 287,288. 291-318,324,326,328,330, etc 292-319,321. 323,325,327,329. 332,335. 334,337. 365-367. 369,370. 372-376. 381,384. 382-385. 403-429. 438-459. 466-487. 489498. 503-509. 512-514. 531-533.

Environmental and Diagnostic Questionnaires : No 80 53 54 55 56 57 58 59

36 69 59 33 11 58 4

HAND YES-NO SPM FAD CONMENTS SPECIALIST ALTERNATIVE

535,537. 536,538,540,543-547,549, etc 553-575. 602-630,644-752. 754-756. 759-762. 763-766.

DNP Questionnaires : Nos. 91 to 94 60 61 62 63 64 65 66 67 68 69

25 26 27 1823 21 24 22 19 20

DNP - RATING DNP - SERVICE DNP - WORSE DNP-BETTER DNP - INPROVED DNP - END DNP - PROJECT DNP - HOMEE DNP - CLINIC DNP - COMMENT

778-781,784,785,796-799, 782,783. 786,787. 788,789. 800,801. 816,817. 822-824. 826,827. 828,829. 830-83)2.

etc

7

136

Dystonia -A comprehensiveand longitudinal study in the North East of England

'phddata. sav' DATA SETS Q're No Q're 00 Q're 10 Q're II Q're 20 Q're 21 Q're 30 Q're 40 Q're 50 Q're 60 Q're 70 Q're 80 Q're 81 Q're 91 Q're 00

Nameof Category Hosp,CRN andStatus ID, Geography, EuroQoland SF36HealthStateQ'res(Ist) EuroQoland SF36HealthStateQ'res(2nd) DemographicProfileQuestionnaire TDS Questionnaire Clinical Profile Questionnaire TorticollisQuestionnaire Psychological ProfileQuestionnaire Impactof DystoniaQuestionnaire PrimaryCarer'sQuestionnaire EnvironmentalQuestionnaire DiagnosticQuestionnaire DystoniaNursePractitionerQuestionnaires End of StudyandWBSVariables

No of variables 12 72 74 103 17 III 12 100 13 19 223 12 69 5

Therefore 745,170 data sets= 885 casesx 842 variables

35 Multiple

Response Sets

$Foctot = Total of Focal Dystonias $Famtot = Total of Family Members $Disttot = Total of Family Distribution $Medtot = Total Medication $Dpdtot = Total Dosageper Day $Whentot = Total of When Medication Taken $Prevtot = Total of PreviousTreatments $Septtot = Total Side Effects from Previous Treatments $Comtot Total Comorbidity $Bodtot Total Sites in the Body $Scbttot Total Side Effects of Bot. Tox. $Phystot Total Physical Illnesses $Yrltot Total Years of Onset of Physical Illnesses $Psyctot Total Psychiatric Illnesses $Trettot Total Treatmentsfor Psychiatric Illnesses $Yr5tot Total Years of Onset of Psychiatric Illnesses $Movetot = Total Types of Movement $Paintot = Total Where Pain $Yeartot = Total Years of SpontaneousImprovement $Longtot = Total Length of SpontaneousImprovement $Parttot = Total of Partial or Complete Improvement $Crittot = Total Criticisms of Study $Stattot = Total of Statementsfrom Carer's Strain Index (CSI) $Commtot-- Total - Comments $Spectot = Total - Specialists $Alttot = Total - Alternative Treatments $Hmhtot = Total HN1HServiceNow SWortot = Total of What Makes SpasmsWorse SBettot = Total of What Makes SpasmsBetter $Imptot = Total - ServiceImproved $Servtot = Total - Servicefrom HMH SWholtot = Total - Whole Project $Hometot = Total - Why Home $Clintot = Total - Why Clinic SOthtot = Total - Other Comments.

137

Dystonia -A comprehensiveand longitudinal study in the North East of England

258 VARIABLE NAMES USED AGE ALEX

ACT ANTI

BEND BCS

BATH BP BENE BURD BEF BOD BCSALL BCSSPE BCSPOS BCSEVA BCSTEN BDI

BOTOX BEST BIOF BDITOT

CRN CHIRO COLA

COMP CUT COUNS CONT COMM

CALM CRIT

CON CSI

COP COM CSITOT CHNG

CURR CHEM

COUN CRAV

CAUS COFF

DATE DIAG

DISC DPD

D DEL

DOWN DOC

DOSE DRUG

DOB DISA

DIFF DISF

DAY DECA

DR DNP

DIST

ESD EYER

EURO EYES

EXT EXCL

EXC ENV

EMB ELD ENVAA to

EMP ENVEB

EVNT

EFF

EYEL

ANX ANXI

A ARMR

ACC ARML

ANY ALG

APP AET ALGA to ALGP

AIM ALT

ACUP

FEM FOC FAM FACER FACEL FACES FOOTR FOOTL FDQ FDQALL FD-QSOCFDQPHY FDQSEI FDQLEI FDQOTH FAD FADA to FADZ GEOG

GEN

GH

GEND

GET

GP

GEST

GAS

HOSP

HEAL

HAD

HOW

HAPP

HOUR

HYPN

HANDR HANDL

HAND

ILL

INC

INJ

IOD

IODTOT

JOIN

JAW

KICK

LIFT

LIM

LESS

LIFE

LOT

LAST

LEGR

LEGI,

LIVE

MOB MOST

M NIENT

MOD MEDI

MH MAJ

MAR MALE MOUTH MOVE

MILE

MINS

MED

MEM

NERV

NOTDYSNUMB

NEWS

NECK

PREV

PHYSIO

OSTATE ONE

ONSET

OSTEO OTHER

PF PAIN PSYCHO PHYS

PART PSYC

PEOP PAIN

POST PSTN

QUAL

RP

RE

RECODE

LOW

NESTEEM

PRIM PAY PESTEEM

SURV SEC SES

STAT SELF SGMT SIDE SESTOT SMOK

S SEPT SOUN

SEV SEBT SUN

SOC SF SUP SITE SHG SURG SPEECH SEVE SPMA to SPM-W SPEC

TIME TRUNK

TIR TRUE

TYPE THY

TRAV TEA

TRIG

TALK UNCO

TDS

TOLD VIG

TRET TONGUE VOCAL VT

WALK WHY

WALB WRIT

WALC WAT

WORK WINE

WORN

WOR X

WHO

WHEN YNG

WHAT YOGA

SEG SPON

WHOM YEAR

138

Home

POST Value

Post

Label

Garden, Herts Welwyn Birmingham Cleckheaton, W Yorks Keighley, W Yorks Lancs Bolton, Lancs Bury, Worthing, Sussex Cumbria Carlisle, Cumbria Carlisle, Carlisle, Cumbria Carlisle, Cumbria Cumbria Carlisle, Carlisle, Cumbria Carlisle, Cumbria Carlisle, Cumbria Scotby, Carlisle Low Hesket, Carlisle Aspatria, Cumbria Linstock, Carlisle Wigton, Cumbria Brampton, Cumbria Cumbria Penrith, Penrith, Cumbria Penrith, Cumbria Penrith, Cumbria Penrith, Cumbria Keswick, Cumbria Cockermouth, Cumbria Cockermouth, Cumbria Workington, Cumbria Workington, Cumbria Workington, Cumbria Workington, Cumbria Workington, Cumbria Maryport, Cumbria Maryport, Cumbria Appleby, Cumbria Kirby Stephen, Cumb. Ravenglass, Cumbria Holmbrook, Cumbria Seascale, Cumbria Cleator Moor, Cumb. Frizington, Cumbria Whitehaven, Cumbria . Whitehaven, Cumbria Whitehaven, Cumbria Whitehaven, Cumbria Gravesend, Kent Montrose, Scotland Dumfries, Scotland Canonbie, Scotland Gretna, Scotland Durham City Durham City Durham City Durham City Durham City Ch Le St, Durham Ch Le St, Durham Ch Le St, Durham Birtley, Ch Le St. Birtley, Ch Le St. Ch Le St, Durham Houghton Le Spring BUrnmoor, H. Le Sp.

Code Frequency

Value AL08 B26 BD19 BD20 BL06 BLO8 BN12 CA01 CA01 CA01 CA02 CA02 CA02 CA03 CA03 CA04 CA04 CA05 CA06 CA07 CA08 CA10 CA10 CAll CAll CAll CA12 CA13 CA13 CA14 CA14 CA14 CA14 CA14 CA15 CA15 CA16 CA17 CA18 CA19 CA20 CA25 CA26 CA28 CA28 CA28 CA28 DA12 DD10 DG02 DG14 DG16 DH01 DH01 DH01 DH01 DH01 DH02 DH02 DH02 DH03 DH03 DH03 DH04 DH04

1 2 3 4 6 7 0 9 0 8 3 4 0 1 1 3 0 8 9 4 0 9 1 2 3 4 5 6 7 6 4 1 1 1 5 3 6 7 8 9

1 2 3 4 5 1 2 3 1 2 4 4 5

Percent 1 1 2 1 1 1 1 1 5 1 2 2 6 1 2 2 1 1 2 1 2 2 1 2 2 1 1 1 2 5 1 1 2 1 1 1 1 2 1 1 3 2 1 1 1 1 2 1 1 1 1 1 2 2 2 2 5 4 1 2 5 2 4 2 1

.1 .1 .2 .1 .1 .1 .1 .1 .5 .1 .2 .2 .6 .1 .2 .2 .1 .1 .2 .1 .2 .2 .1 .2 .2 .1 .1 .1 .2 .5 .1 .1 .2 .1 .1 .1 .1 .2 .1 .1 .3 .2 .1 .1 .1 .1 .2 .1 .1 .1 .1 .1 .2 .2 .2 .2 .5 .4 .1 .2 .5 .2 .4 .2 .1

Valid Percent .1 .1 .2 .1 .1 .1 .1 .1 .5 .1 .2 .2 .6 .1 .2 .2 .1 .1 .2 .1 .2 .2 .1 .2 .2 .1 .1 .1 .2 .5 .1 .1 .2 .1 .1 .1 .1 .2 .1 .1 .3 .2 .1 .1 .1 .1 .2 .1 .1 .1 .1 .1 .2 .2 .2 .2 .5 .4 .1 .2 .5 .2 .4 .2 .1

Cum Percent .1 .2 .4 .5 .6 .7 .9 1.0 1.5 1.6 1.6 2.0 2.7 2.8 3.0 3.2 3.3 3.4 3.6 3.7 3.9 4.2 4.3 4.5 4.7 4.8 4.9 5.0 5.2 5.8 5.9 6.0 6.2 6.3 6.4 6.5 6.6 6.8 6.9 7.0 7.4 7.6 7.7 7.8 7.9 8.0 8.2 8.3 8.4 8.5 8.6 8.8 9.0 9.2 9.4 9.6 10.1 10.6 10.7 10.9 11.4 11.6 12.1 12.3 12.4

Home Post

POST Value

Label

H. Le Sp. Fenceho, H. Le Sp. Penshaw, Le Hole Hetton Le Spring Houghton E Rainton, Durham Durham Sherburn, Coll, Shotton 'Durham Wheatley, Durham Coxhoe, Durham Durham Bowburn, Lanchester, Durham Sacriston, Durham Langley Park, Durham Consett, Co. Durham Consett, Co. Durham Consett, Co. Durham Stanley, Co. Durham Stanley, Co. Durham Stanley, Co. Durham Anfield Plain, Co. D Dipton, Stanley Darlington, Co. D Darlington, Co. D Darlington, Co. D Darlington, Co. D Darlington, Co. D M. St. G, Co. Durham Hurworth, Co. Durham Darlington, Co. D Darlington, Co. D Darlington, Co. D Darlington, Co. D Shildon, Co. D Shildon, Co. D Newton Ay., Co. D Newton Ay., Co. D School Ay., Co. D Newton Ay., Co. D N'allerton, N. Yorks Osmotherley, N Yorks NIallerton, N. Yorks N'allerton, N. Yorks Bedale, N. Yorks Leyburn, N. Yorks Catterick, N. Yorks Gunn. Richmond, N. Yks B Castle, Durham B Castle, Durham Stanhope, B Castle Wolsingham, Co. D Tow Law, Co. D B Auckland, Co. D B Auckland, Co. D B Auckland, Co. D B Auckland, Co. D B Auckland, Co. D Crook, Co. Durham Crook, Co. Durham Spennymoor, Durham Spennymoor, Durham Ferryhill,, Co Durham Ferryhill, Co Durham Ferryhill, Co Durham Doncaster, S Yorks. Scunthorpe, Lincs Cinderford, Glos

Code Value DH04 6 DH04 7 DHO5 0 DHO5 8 DH05 9 DHO6 1 DH06 2 DH06 3 DH06 4 DH06 5 DH07 0 DH07 6 DH07 9 DHO8 0 DHOB 6 DH08 7 DH09 0 DH09 6 DHO9 7 DHO9 8 DH09 9 DLO1 1 DLO1 2 DLO1 3 DLO1 4 DLO1 5 DL02 1 DL02 2 DL03 0 DL03 6 DL03 8 DL03 9 DL04 1 DL04 2 DLO5 4 DL05 5 DL05 6 DL05 7 DL06 2 DL06 3 DL07 8 DL07 9 DLO8 2 DL09 3 DLIO 7 DL11 6 DL12 8 DL12 9 DL13 2 DL13 3 DL13 4 DL14 0 DL14 6 DL14 7 DL14 8 DL14 9 DL15 0 DL15 9 DL16 6 DL16 7 DL17 0 DL17 8 DL17 9 DN07 DN17 GL14

Frequency 1 3 1 2 2 2 4 3 4 3 1 7 4 1 1 1 1 5 1 1 1 6 8 2 8 2 2 4 3 4 7 3 1 5 3 2 2 3 2 1 1 1 2 1 1 1 3 3 1 1 1 1 3 1 2 3 3 5 3 4 1 4 2 2 1 1

Percent .1 .3 .1 .2 .2 .2 .4 .3 .4 .3 .1 .7 .4 .1 .1 .1 .1 .5 .1 .1 .1 .6 .9 .2 .9 .2 .2 .4 .3 .4 .7 .3 .1 .5 .3 .2 .2 .3 .2 .1 .1 .1 .2 .1 .1 .1 .3 .3 .1 .1 .1 .1 .3 .1 .2 .3 .3 .5 .3 .4 .1 .4 .2 .2 .1 .1

Valid Percent .1 .3 .1 .2 .2 .2 .4 .3 .4 .3 .1 .7 .4 .1 .1 .1 .1 .5 .1 .1 .1 .6 .9 .2 .9 .2 .2 .4 .3 .4 .7 .3 .1 .5 .3 .2 .2 .3 .2 .1 .1 .1 .2 .1 .1 .1 .3 .3 .1 .1 .1 .1 .3 .1 .2 .3 .3 .5 .3 .4 .1 .4 .2 .2 .1 .1

Cum Percent 12.5 12.8 12.9 13.1 13.3 13.6 14.0 14.3 14.7 15.0 15.2 15.9 16.3 16.4 16.5 16.6 16.8 17.3 17.4 17.5 17.6 18.2 19.1 19.3 20.2 20.4 20.6 21.0 21.3 21.8 22.5 22.8 22.9 23.5 23.8 24.0 24.2 24.5 24.8 24.9 25.0 25.1 25.3 25.4 25.5 25.6 25.9 26.3 26.4 26.5 26.6 26.7 27.0 27.1 27.3 27.6 28.0 28.5 28.8 29.2 29.3 29.8 30.0 30.2 30.3 30.4

POST Value

Home

Post

Label

Farnborough, Hants Middx Harrow, Lewis Stornoway, Hull Springfield, Sutton-on-Hull Hull Woodmansey, W Yorks Halifax, C. I. Jersey, Milnthorpe, Cumbria Cumbria Kendal, Cumbria Kendal, Cumbria Kendal, Cumbria Cartmell, Ulverston, Cumbria in Furness Barrow in Furness Barrow in Dalton Furness Rillom, Cumbria Ambleside, Cumbria Ambleside, Cumbria Windermere, Cumbria Wales Colwyn, Clwyd, Lincoln Leeds Leeds Leeds Leeds Leeds Manchester Newcastle Tyne upon Newcastle Tyne upon Newcastle Tyne upon Newcastle Tyne upon Gosforth, Newcastle Ouseburn, Newcastle N Kenton, Newcastle Kenton, Newcastle Gosforth, Newcastle Elswick, Newcastle Cruddas, Newcastle Grainger, Newcastle Fenham, Newcastle Chapel Pk, Newcastle Fenham, Newcastle Blakelaw, Newcastle Newbiggin, Newcastle Byker, Newcastle Walker, Newcastle Bensham, Newcastle Heaton, Newcastle High Heaton, Newc. Gateshead, T&W Teams, Gateshead Deckham, Gateshead Bensham, Gateshead Sheriff Hill, Gates. Low Fell, Gateshead Gateshead, T&W Pelaw, Gateshead Wardley, Gateshead Felling, Gateshead Lobley Hill, Gates. Dunston, Gateshead Forest Hall, N Tyne Longbenton, N Tyne Palmersville, N Tyne

Code Value GU14 RA02 HS01 HU05 HU07 HU17 HX02 JE03 LA07 LA08 LA09 LA09 LA11 LA12 LA13 LA14 LA15 LA18 LA22 LA22 LA23 LL29 LN04 LS05 LS07 LS08 LS12 LS17 M28 NE01 NE02 NE02 NE02 NE03 NE03 NE03 NE03 NE03 NE04 NE04 NE04 NE04 NE05 NE05 NE05 NE05 NE06 NE06 NE06 NE06 NE07 NE08 NE08 NE08 NE08 NE09 NE09 NE09 NE10 NE10 NE10 NE11 NE11 NE12 NE12 NE12

Frequency

7 8 5 7 6 0 9 4 8 5 0 9 2

8 1 3 4 1 2 3 4 5 6 7 8 9 1 2 3 4 2 3 4 5 7 0 2 3 4 5 6 7 0 8 9 0 9 0 8 9

Percent 1 1 1 1 1 1 1 1 1 1 1 3 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 3 2 2 9 4 3 3 2 2 2 2 2 4 4 4 1 4 2 7 2 5 1 2 3 6 4 4 2 3 7 3 1 2 4 4 3

Valid Percent

.1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .3 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .3 .2 .2 1.0

.1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .3 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .3 .2 .2 1.0

.4 .3 .3 .2 .2 .2 .2 .2 .4 .4 .4 .1 .4 .2 .7 .2 .5 .1 .2 .3 .6 .4 .4 .2 .3 .7 .3 .1 .2 .4 .4 .3

.4 .3 .3 .2 .2 .2 .2 .2 .4 .4 .4 .1 .4 .2 .7 .2 .5 .1 .2 .3 .6 .4 .4 .2 .3 .7 .3 .1 .2 .4 .4 .3

Cum Percent 30.5 30.6 30.7 30.8 30.9 31.1 31.2 31.3 31.4 31.5 31.6 31.9 32.0 32.1 32.2 32.3 32.4 32.6 32.7 32.8 32.9 33.0 33.1 33.2 33.3 33.4 33.5 33.6 33.7 33.8 34.2 34.4 34.6 35.5 36.0 36.3 36.6 36.8 37.0 37.2 37.5 37.7 38.1 38.5 39.0 39.1 39.5 39.7 40.4 40.7 41.2 41.3 41.5 41.8 42.5 42.9 43.3 43.5 43.9 44.6 44.9 45.0 45.3 45.7 46.1 46.4

Home

POST Value

Post

Label

Burn, Seaton N Tyne Wideopen, Newcastle Horsley, Newcastle Pendower, Newcastle Newby, Newcastle Lemington, Newcastle Throckley, Newcastle Swallwell, Newcastle Whickham, Newcastle Marley Hill, Newc. Burnopfield, Co. D Chopwell, Gateshead Otterburn, N'land Kirkheaton, N'land Ponteland, N'land Blaydon, T&W Blaydon, T&W Winlaton, T&W Bedlington, N'land Cramlington, N'land Cramlington, N'land Cramlington, N'land Cramlington, N'land Blyth, N'land Blyth, N'land Blyth, N'land Whitley Bay, T&W Whitley Bay, T&W Whitley Bay, T&W Whitley Bay, T&W Whitley Bay, T&W Backwood, Newcastle Howden, Wallsend Wallsend, T&W Holycross, Wallsend High Farm, Wallsend Hadrian Pk, Wallsend North Shields, T&W New York, N Shields North Shields, T&W North Shields, T&W North Shields, T&W North Shields, T&W Hebburn, S Tyne Hebburn, S Tyne Jarrow, S Tyne Jarrow, S Tyne S Shields, S Tyne S Shields, S Tyne S Shields, S Tyne S Shields, S Tyne S Shields, S Tyne S Shields, S Tyne S Shields, S Tyne S Shields, S Tyne Boldon Coll, S Tyne East Boldon and West Donwell, Washington Washington, T&W Washington, T&W Ayton, Washington Biddick, Washington Fatfield, Washington High spen, T&W Ryton, T&W Crawcrook, Ryton

Code Val ue NE13 NE13 NE15 NE15 NE15 NE15 NE15 NE16 NE16 NE16 NE16 NE17 NE19 NE19 NE20 NE21 NE21 NE21 NE22 NE23 NE23 NE23 NE23 NE24 NE24 NE24 NE25 NE25 NE25 NE26 NE26 NE27 NE28 NE28 NE28 NE28 NE28 NE29 NE29 NE29 NE30 NE30 NE30 NE31 NE31 NE32 NE32 NE33 NE33 NE33 NE34 NE34 NE34 NE34 NE34 NE35 NE36 NE37 NE37 NE37 NE38 NE38 NE38 NE39 NE40 NE40

6 7 0 6 7 8 9 3 4 5 6 7 1 2 9 4 5 6 5 6 7 8 9 2 3 5 0 8 9 3 4 0 0 6 7 8 9 0 8 9 1 2 3 1 2 4 5 2 3 5 0 6 7 8 9 9 0 1 2 3 0 7 8 2 3 4

Frequency 3 2 1 3 4 3 3 1 2 2 1 2 1 1 1 2 2 2 2 5 4 1 5 1 2 4 4 4 7 4 2 3 1 1 1 3 1 1 2 2 1 2 5 1 4 2 4 1 2 1 3 1 7 5 1 3 2 2 2 1 2 4 3 1 5 3

Percent .3 .2 .1 .3 .4 .3 .3 .1 .2 .2 .1 .2 .1 .1 .1 .2 .2 .2 .2 .5 .4 .1 .5 .1 .2 .4 .4 .4 .7 .4 .2 .3 .1 .1 .1 .3 .1 .1 .2 .2 .1 .2 .5 .1 .4 .2 .4 .1 .2 .1 .3 .1 .7 .5 .1 .3 .2 .2 .2 .1 .2 .4 .3 .1 .5 .3

Valid Percent .3 .2 .1 .3 .4 .3 .3 .1 .2 .2 .1 .2 .1 .1 .1 .2 .2 .2 .2 .5 .4 .1 .5 .1 .2 .4 .4 .4 .7 .4 .2 .3 .1 .1 .1 .3 .1 .1 .2 .2 .1 .2 .5 .1 .4 .2 .4 .1 .2 .1 .3 .1 .7 .5 .1 .3 .2 .2 .2 .1 .2 .4 .3 .1 .5 .3

Cum Percent 46.7 47.0 47.1 47.4 47.8 48.1 48.5 48.6 48.8 49.0 49.1 49.3 49.4 49.5 49.6 49.8 50.1 50.3 50.5 51.0 51.4 51.5 52.1 52.2 52.4 52.8 53.3 53.7 54.4 54.9 55.1 55.4 55.5 55.6 55.7 56.0 56.1 56.2 56.5 56.7 56.8 57.0 57.5 57.6 58.1 58.3 58.7 58.8 59.0 59.1 59.4 59.6 60.3 60.8 60.9 61.3 61.5 61.7 61.9 62.0 62.2 62.6 63.0 63.1 63.6 63.9

Home Post

POST Value

Label

N'land Prudhoe, N'land Prudhoe, Mill, Riding N'land N'land Corbridge, Hexham, N'land Hexham, N'land Haydon Br., N'land Hexham Allendale, Haltwhistle, N'land N'land Morpeth, Morpeth, N'land Choppington, N'land Ashington, N'land N'land Ashington, Ashington, N'land Newbiggin, N'land Amble, N'land Hepple, Morpeth Longhorsley, Morpeth Felton, Morpeth Alnwick, N'land Bolton, Alnwick Bamburgh, N'land Mansfield, Notts Norwich, Norfolk Plymouth, Devon Totland, 1.0. W. Wokingham, Berks Bracknell, Berks Sheffield Sheffield Rotherham, S Yorks Barnsley, W Yorks W Norwood, London Buxton, Lancs Hendon, S'land Ashbrooke, S'land Hendon, Sunderland Grangetown, S'land Silksworth, S'land Doxford, Sunderland Herrington, S'land Springfield, S'land S Hylton, Sunderland Millfield, S'land Sunderland, T&W High Barnes, S'land Grindon, Sunderland Fulwell, Sunderland Southwick, S'land Hylton Castle S'land Town End Farm S'land Witherwack, S'land Monkwearmouth S'land Whitburn, Sunderland Seaburn Dene, S'land Fulwell, Sunderland Seaham, Co. Durham Seaham, Co. Durham SeahaM. Co. Durham Peterlee, Co. Durham Peterlee, Co. Durham Peterlee, Co. Durham Peterlee, Co. Durham Peterlee, Co. Durham Duns, Berwickshire

Code Val ue NE42 NE42 NE44 NE45 NE46 NE46 NE47 NE47 NE49 NE61 NE61 NE62 NE63 NE63 NE63 NE64 NE65 NE65 NE65 NE65 NE66 NE66 NE69 NG20 NR05 PL07 P039 RG11 RG12 S21 S25 S65 S73 SE27 SK17 SR01 SR02 SR02 SR02 SR03 SR03 SR03 SR03 SR04 SR04 SR04 SR04 SR04 SR05 SR05 SR05 SR05 SR05 SR06 SR06 SR06 SR06 SR07 SR07 SR07 SR08 SR08 SR08 SR08 SR08 TD11

5 6 6 5 1 2 6 9 9 2 5 5 0 8 9 6 0 7 8 9 1 2 7

2 7 8 9 1 2 3 4 0 6 7 8 9 1 2 3 4 5 0 7 8 9 0 8 9 1 2 3 4 5

Frequency 4 2 1 1 2 2 1 2 2 1 3 2 2 2 5 1 1 1 1 2 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 1 2 1 2 7 5 3 3 3 1 5 2 3 2 1 2 2 5 1 2 3 3 2 3 2 1 4 1 1 2 1

Percent .4 .2 .1 .1 .2 .2 .1 .2 .2 .1 .3 .2 .2 .2 .5 .1 .1 .1 .1 .2 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .2 .1 .2 .7 .5 .3 .3 .3 .1 .5 .2 .3 .2 .1 .2 .2 .5 .1 .2 .3 .3 .2 .3 .2 .1 .4 .1 .1 .2 .1

Valid Percent .4 .2 .1 .1 .2 .2 .1 .2 .2 .1 .3 .2 .2 .2 .5 .1 .1 .1 .1 .2 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .1 .2 .1 .2 .7 .5 .3 .3 .3 .1 .5 .2 .3 .2 .1 .2 .2 .5 .1 .2 .3 .3 .2 .3 .2 .1 .4 .1 .1 .2 .1

Cum Percent 64.4 64.6 64.7 64.8 65.0 65.2 65.3 65.5 65.7 65.8 66.2 66.4 66.6 66.8 67.3 67.4 67.6 67.7 67.8 68.0 68.1 68.2 68.3 68.4 68.5 68.6 68.7 68.8 68.9 69.1 69.2 69.3 69.4 69.5 69.6 69.7 69.9 70.0 70.2 71.0 71.5 71.8 72.1 72.5 72.6 73.1 73.3 73.6 73.9 74.0 74.2 74.4 74.9 75.0 75.2 75.6 75.9 76.1 76.4 76.6 76.7 77.2 77.3 77.4 77.6 77.7

Home Post

POST Value

Label

Berwick upon Tweed Berwick upon Tweed Cornwall Penzance, Middlesbrough Middlesbrough middlesbrough Middlesbrough Pk, MIbro Pallister N Ormesby, MIbro Hills, Berwick MIbro Pk, MIbro Pallister Middlesbrough Middlesbrough Linthorpe, M'Bro Acklam, M'Bro Acklam, M'Bro Acklam, M'Bro Normanby, M'Bro South Bank, M'Bro Grangetown, M'Bro Eston, M'Bro Nunthorpe, M'Bro Marton, M'Bro M'Bro Ormesby, Coulby Newham, M'Bro Hemlington, M'Bro Stokesley, M'Bro Great Ayton, M'Bro Redcar in Cleveland Dalton Park, Redcar Redcar in Cleveland Redcar in Cleveland Marske-by-the-sea Marske-by-the-sea Saltburn-by-the-sea Skelton, Saltburn Loftus, Saltburn Runswick Bay, N Yks Guisborough, Cleve. Guisborough, Cleve. Hutton Rudby, Yarm Yarm, Stockton Eaglescliffe, Stock Egglescliffe, Stock Thornaby, Stockton Ingleby Barwick, St. Thornaby, Stockton Thornaby, Stockton Stockton-on-Tees Stockton-on-Tees Oxbridge, Stockton Hartburn, Stockton Stockton-on-Tees Newtown, Stockton Fairfield, Stockton Hardwick, Stockton Ragworth, Stockton Norton, Stockton Norton, Stockton Stillington, Co. D Sedgefield, Co. D Sedgefield, Co. D Fishburn, Stockton Stillington, Co. D. Billingham, Stockton Billingham, Stockton

Code Frequency

Value TD15 TD15 TR18 TS01 TS01 TS01 TS01 TS03 TS03 TS03 TS03 TS04 TS04 TS05 TS05 TS05 TS05 TS06 TS06 TS06 TS06 TS07 TS07 TS07 TS08 TS08 TS09 TS09 TS10 TS10 TS10 TS10 TS11 TS11 TS12 TS12 TS13 TS13 TS14 TS14 TS15 TS15 TS16 TS16 TS17 TS17 TS17 TS17 TS18 TS18 TS18 TS18 TS19 TS19 TS19 TS19 TS19 TS20 TS20 TS21 TS21 TS21 TS21 TS21 TS22 TS23

1 2 2 3 4 5 0 6 7 8 2 3 5 6 7 8 0 6 7 9 0 8 9 0 9 5 6 1 2 3 4 6 7 1 2 4 5 6 7 0 9 0 9 0 5 8 9 1 3 4 5 0 6 7 8 9 1 2 1 2 3 4 7 5 2

1 2 1 1 1 2 1 3 3 2 3 4 3 2 3 1 7 3 2 4 5 2 4 4 4 4 1 1 1 1 4 1 2 1 7 6 2 1 4 4 1 2 3 1 4 1 3 1 1 1 1 3 5 1 5 1 1 2 6 1 1 3 1 1 3 2

Percent .1 .2 .1 .1 .1 .2 .1 .3 .3 .2 .3 .4 .3 .2 .3 .1 .7 .3 .2 .4 .5 .2 .4 .4 .4 .4 .1 .1 .1 .1 .4 .1 .2 .1 .7 .6 .2 .1 .4 .4 .1 .2 .3 .1 .4 .1 .3 .1 .1 .1 .1 .3 .5 .1 .5 .1 .1 .2 .6 .1 .1 .3 .1 .1 .3 .2

Valid Percent .1 .2 .1 .1 .1 .2 .1 .3 .3 .2 .3 .4 .3 .2 .3 .1 .7 .3 .2 .4 .5 .2 .4 .4 .4 .4 .1 .1 .1 .1 .4 .1 .2 .1 .7 .6 .2 .1 .4 .4 .1 .2 .3 .1 .4 .1 .3 .1 .1 .1 .1 .3 .5 .1 .5 .1 .1 .2 .6 .1 .1 .3 .1 .1 .3 .2

Cum Percent 77.8 78.0 78.1 78.2 78.3 78.5 78.7 79.0 79.3 79.5 79.8 80.3 80.6 80.8 81.1 81.2 82.0 82.3 82.5 82.9 83.5 83.7 64.1 84.5 85.0 85.4 85.5 85.6 85.7 85.8 86.2 86.3 86.6 86.7 87.4 88.0 88.3 88.4 88.8 89.2 89.3 89.5 89.9 90.0 90.4 90.5 90.8 90.9 91.0 91.1 91.2 91.6 92.1 92.2 92.7 92.8 93.0 93.2 93.8 93.9 94.0 94.3 94.5 94.6 94.9 95.1

Home

POST Value

Post

Label

Code

Billingham, Stockton Hartlepool, Cleve. Hartlepool, Cleve. Hartlepool, Cleve. Hartlepool, Cleve. Hartlepool, Cleve. Hartlepool, Cleve. Hartlepool, Cleve. Hartlepool, Cleve. Hartlepool, Cleve. Hartlepool, Cleve. Coll Blackhall Co. D Wingate, Co. Durham Trimdon, Co. Durham Middx Twickenham, Wakefield, W Yorks Wakefield, W Yorks W Yorks ossett, Pontefract, W Yorks Castleford, W Yorks City of York Scarborough, N Yorks Filey, N Yorks Pickering, York Wheldrake, York Whitby, N Yorks Whitby, N Yorks Acomb, York Langtoft, N Yorks

TS23 TS24 TS24 TS24 TS25 TS25 TS25 TS26 TS26 TS26 TS27 TS27 TS28 TS29 TW02 WF03 WF04 WF05 WFOS WFIO Y003 Y013 Y014 Y018 Y019 Y021 Y022 Y024 Y025 Tota

Valid

cases

Frequency

Value

937

Missing

3 0 8 9 3 4 5 0 8 9 3 4 5 6

Percent 1 4 1 1 2 2 6 1 1 3 2 2 1 2 1 1 1 2 1 1 1 1 1 1 1 2 1 1 1

.1 .4 .1 .1 .2 .2 .6 .1 .1 .3 .2 .2 .1 .2 .1 .1 .1 .2 .1 .1 .1 .1 .1 .1 .1 .2 .1 .1 .1

-------

l

------100.0

937 cases

0

Valid Percent .1 .4 .1 .1 .2 .2 .6 .1 .1 .3 .2 .2 .1 .2 .1 .1 .1 .2 .1 .1 .1 .1 .1 .1 .1 .2 .1 .1 .1 ------100.0

Cum Percent 95.2 95.6 95.7 95.8 96.1 96.3 96.9 97.0 97.1 97.4 97.7 97.9 98.0 98.2 98.3 98.4 98.5 98.7 98.8 98.9 99.0 99.1 99.3 99.4 99.5 99.7 99.8 99.9 100.0

"PHD-GEOG. TITLE 'updated SUBTITLE RECODE (ICA01 (ICA. 02 (ICA03 (ICA04 (ICA05 (ICA06 (ICA07 (ICA08 (ICA. 10 (ICAll (ICA. 12 (ICA14 (ICA15 (ICA18 (ICA. 23 (ICA. 28 (IDH01 (IDHO2 (IDHO3 (IDHO4 (IDHO5 (IDHO6 (IDHO7 (IDHO8 (IDHO9 (IDLO1 (IDLO2 (IDLO3 (IDLO4 (IDLO5 (lDL06 (IDLO7 (IDLO8 (IDLO9 (IDL10 (IDL11 (IDL12 (IDL13 (IDL14 (IDL15 PDL16 (IDL17 (INE01 ý(INE01 ('NE02 (INE03 ('NE04 PNE05 PNE06 PNE07 PNE09 PNE10 (INE11 PNE13 PNE15 PNE16 PNE17 PNE20 PNE21 PNE22 PNE23 PNE24 PNE25 PNE26 PNE28

post 11=50) 41=50) 81=50) 81=50) 11=50) 41=50) 81=50) 11=50) 11=50) 71=50) 41=50) 11=50) 61=50) 11=60) 31=60) 61=60) 11=10) 11=10) 11=30) 41=30) 81=30) 11=10) 61=10) 51=10) 61=10) 11=10) 11=10) 61=10) 11=10) 41=10) 11=70) 81=70) 11=70) 31=70) 41=70) 61=70) 81=10) 11=10) 61=10) 81=10) 61=10) 81=10) 11=30) 61=30) 11=30) 11=30) 51=30) 11=30) 11=30) 71=30) 51=30) 81=30) 91=30) 61=30) 61=30) 31=30) 71=30) 91=40) 41=30) 51=40) 61=40) 11=40) 81=30) 11=30) 61=30)

SPS = SYNTAX FILE FOR ESD / SER / PSR / DNP / PHD" FROM POST CODES' as of 19.11.9B - BOUNDARIES

(ICA. 01 (ICA02 (ICA. 03 (ICA04 (ICA05 (ICA06 (ICA. 07 (ICA08 (ICA10 (ICAll (ICA12 PCA14 (ICA15 (ICA19 PCA. 24 (ICA. 28 (IDH01 (IDHO2 (IDHO3 (IDHO4 (IDHO5 (IDHO6 (IDHO7 (lDH08 (IDHO9 (IDLO1 (IDLO2 (IDLO3 (IDLO4 (lDL05 (IDLO6 (IDLO7 (IDLO8 (IDLO9 (IDL10 (IDL11 (IDL12 (IDL13 (IDL14 (IDL15 (IDL16 (IDL17 (INE01 (INE01 (INE02 (INE03 (INE04 (INE05 (INE06 (INE08 (INE09 (INE10 (INE11 (INE13 (INE15 (INE16 (INE18 (INE20 (INE21 (INE22 (INE23 (INE24 (INE25 (INE26 (INE28

2=50) 51=50) 91=50) 9'=50) 21=50) 51=50) 91=50) 21=50) 21=50) 81=50) 51=50) 21=50) 71=50) 11=60) 31=60) 71=60) 21=10) 21=10) 2'=30) 51=30) 91=30) 21=10) 71=10) 61=10) 71=10) 2'=10) 21=10) 71=10) 21=10) 51=10) 21=70) 91=70) 21=70) 4'=70) 51=70) 7'=70) 91=10) 21=10) 71=10) 91=10) 71=10) 91=10) 2'=30) 71=30) 2'=30) 2'=30) 61=30) 21=30) 21=30) 11=30) 61=30) 91=30) 01=30) 71=30) 71=30) 41=30) 01=40) 01=40) 51=30) 61=40) 71=30) 21=40) 91=30) 21=30) 71=30)

('CA01 (ICA02 (ICA03 ('CA04 (ICA05 (ICA06 (ICA07 (ICA. 09 (ICA. 10 (ICAll (ICA13 (ICA14 (ICA15 (ICA. 20 (ICA. 25 (ICA28 (IDH01 (IDHO2 ('DH03 (IDHO4 (IDHO5 (IDHO6 (IDHO7 (lDH08 (IDHO9 (IDLO1 (IDLO2 (IDI. 03

3'=50) 61=50) 01=50) 0'=50) 31=50) 61=50) 01=50) 31=50) 31=50) 91=50) 91=50) 31=50) 81=50) 11=60) 51=60) 81=60) 31=10) 31=10) 3'=10) 61=10) 01=30) 31=10) 81=10) 71=10) 81=10) 31=10) 31=10) 81=10)

(IDLO5 (IDLO6 (lDL07 (lDL08

61=10) 31=70) 01=70) 31=70)

(IDL10

(ICA02

71=50)

('CA05 (ICA05 (ICA06

61=50) 41=50) 71=50)

(IcAll (ICA13 (ICA. 14 (ICA16 (ICA21 (ICA. 26 (ICA28 (IDH01

01=50) 01=50) 41=50) 61=50) 21=60) 31=60) 91=60) 41=10)

('DH03 (IDHO4 (IDHO8 (IDHO6 (IDHO7 (IDHO8 (IDHO9 (IDLO1

(ICA. 02

81=50)

('CA05 (ICA. 05

7'=50) 51=50)

(ICA14 (ICA17 (ICA22 (ICA27

51=50) 4, =50) 21=60) 01=60)

(IDH01

51=10)

41=10) 71=30) 01=10) 41=10) 91=10) 81=10) 91=10) 41=10)

(IDHO6 (IDHO7 ('DH08 (IDHO9 (IDLO1

5'=10) 01=10) 9'=10) 01=10) 51=10)

(IDLO3

91=10)

(IDLO3

0, =10)

(IDLO5

71=10)

(IDLO8

41=60)

(IDLOS

51=60)

61=70)

(IDL10

71=70)

(IDL12 (IDL13 (IDL14 (IDL15

0'=10) 31=10) 81=10) 01=10)

(IDL13 (IDL14

41=10) 91=10)

(IDL13 (IDL14

5, =10) 01=10)

(IDL17 ý'NE01 (INE01 ('NE02 ('NE03 (INE04 (INE05 (INE06 (INE08 (INE09 (INE10 (INE12 (INE13 (INE15 (INE16 (INE19

01=10) 31=30) 81=30) 3'=30) 3'=30) 71=30) 31=30) 31=30) 21=30) 71=30) 01=30) 81=30) 81=30) 81=30) 51=30) 11=40)

(INE01

41=30)

('NE01

5'=30)

('NE02 ('NE03 (INE04 (INE05 (INE06 (INE08

4'=30) 41=30) 81=30) 41=30) 41=30) 31=30)

('NE03 (INE04 (INE05 (INE06 (INE08

5'=30) 9, =30) 5, =30) 51=30) 41=30)

(INE12

91=30)

(INE12

01=30)

(INE15 (INE16 (INE19

91=30) 61=10) 21=40)

(INE15

01=30)

(INE21 (INE22 (INE23 (INE24 (INE25 (INE26 (INE28

61=30) 71=40) 8'=40) 31=40) 01=40) 31=30) 81=30)

('NE23 (INE24

91=40) 41=40)

(INE24

51=40)

(INE26 (INE28

41=40) 91=30)

(INE27 (INE28

01=30) 01=30)

6'=30) ('NE29 ('NE30 V=30) ('NE31 1'=30) ('NE32 3'=30) ('NE33 1'=30) 6'=30) ('NE34 ('NE35 9'=30) ('NE37 1'=30) ('NE38 7'=30) V=30) ('NE39 ('NE41 8'=40) 6'=40) ('NE44 ('NE46 1'=40) ('NE47 5'=40) ('NE47 0'=40) 9'=40) ('NE49 ('NE61 1'=40) 6'=40) ('NE61 ('NE63 8'=40) ('NE65 7'=40) ('NE66 V=40) ('NE67 5'=40) ('SR01 V=30) ('SR02 7'=30) ('SR03 V=30) ('SR04 6'=30) ('SR05 1'=30) ('SR06 7'=30) ('SR07 7'=10) ('SR08 1'=10) ('TD111=90) ('TS01 V=20) PTS03 6'=20) ('TS04 2'=20) PTS05 4=20) PTS06 6=20) (ITS07 81=20) ('TS08 9'=20) PTS10 V=20) (ITS11 61=20) (ITS12 11=20) PTS13 4'=20) PTS15 9'=20) ('TS17 6'=20) PTS18 V=20) (ITS19 71=20) PTS20 V=20) (ITS21 11=10) PTS22 5'=20) PTS24 7'=20) PTS25 V=20) PTS26 8'=20) PTS27 3'=20) PLA05 9=60) PLA07 7'=60) PLA09 4'=60) PLA10 5'=60) PLA12 7'=60) PLA13 9'=60) PLA14 V=60) PLA15 8'=60) PLA17 7=60) PLA20 6=60) PLA23 V=60) PY0031=70) ('AL08'=90) ('DA12'=90) ('DN17'=90) ('HU07'=80) ('LSOBI=80)

('NE29 7'=30) ('NE29 ('NE30 2'=30) ('NE30 ('NE31 2'=30) ('NE32 4'=30) (INE32 PNE33 2'=30) PNE33 ('NE34 7'=30) PNE34 ('NE36 0'=30) ('NE37 2'=30) ('NE37 ('NE38 8'=30) PNE38 ('NE39 2'=30) ('NE40 ('NE42 5'=40) ('NE42 ('NE45 5'=40) ('NE46 2=40) ('NE46 ('NE47 6'=40) ('NE47 ('NE48 V=40) ('NE48 PNE49 0'=40) ('NE61 2'=40) ('NE61 ('NE62 5'=40) ('NE63 9'=40) ('NE63 ('NE65 8'=40) ('NE65 ('NE66 2'=40) ('NE66 ('NE68 7'=40) ('NE69 ('SR01 2'=30) ('SR01 ('SR02 8'=30) ('SR02 PSR03 2'=30) PSR03 ('SR04 7'=30) ('SR04 ('SR05 2'=30) (ISR05 ('SR06 8'=30) ('SR06 ('SR07 8'=10) PSR07 ('SR08 2'=10) ('SROS PTD12 4'=40) ('TD15 ('TS01 2'=20) ('TS01 ('TS03 7'=20) ('TS03 ('TS04 3'=20) ('TS02 ('TS05 5'=20) ('TS05 (TS06 7'=20) ('TS06 ('TS07 9'=20) ('TS07 ('TS08 0'=20) ('TS09 ('TS10 2'=20) ('TS10 ('TS11 71=20) ('TS11 ('TS12 2'=20) (ITS12 ('TS13 51=20) (ITS14 PTS15 0'=20) ('TS16 ('TS17 7'=20) ('TS17 ('TS18 2'=20) PTS18 ('TS19 8'=20) ('TS19 PTS20 2'=20) ('TS21 2'=10) ('TS21 PTS23 V=20) ('TS23 ('TS24 8'=20) ('TS24 ('TS25 2'=20) ('TS25 ('TS26 9'=20) ('TS26 ('TS27 4'=10) ('TS28 (ILA05 01=60) ('LA06 ('LA08 8'=60) ('LA08 MA09 5=60) ('LA09 MAll 6'=60) (ILAll PLA12 8'=60) ('LA12 MA13 0'=60) ('LA14 2=60) (ILA14 (LA16 71=60) MA18 4'=60) ('LA18 MA21 8'=60) (ILA22 MA23 2'=60) ('IA23 ('YO04'=70) ('YO13'=70) ('B26'=90) ('BD19'=90) ('DD10'=90) ('DG02'=90) ('GL14'=90) ('GU14'=90) ('HU17'=80) ('HX02'=80) (LS12'=80) ('LS17'=80)

8'=30) 3'=30)

PNE29 ('NE30

9'=30) 4'=30)

('NE29

0'=30)

5'=30) 3'=30) 8'=30)

('NE33 ('NE34

4'=30) 9'=30)

('NE33 ('NE34

5'=30) 0'=30)

3'=30) 9'=30) 3'=30) 6'=40)

('NE38 PNE40 ('NE43

0'=30) 4'=30) 7'=40)

3'=40) 7'=40) 2'=40)

('NE46 ('NE47 ('NE48

4'=40) 8'=40) 3'=40)

('NE47 ('NE48

9'=40) 4'=40)

3'=40)

PNE61

4'=40)

('NE61

5'=40)

0'=40) 9'=40) 3'=40) 7'=40) 3'=30) 9'=30) 3'=30) 8'=30) 31=30) 9'=30) 91=10) 3'=10) V=40) 3'=20) 8'=20) V=20) 6'=20) 8'=20) 0'=20) 5'=20) 3'=20) 8'=20) 3'=20) 6'=20) 9'=20) 8'=20) 3'=20) 9'=20)

PNE64 ('NE65 ('NE66 ('NE70

6'=40) 0'=40) 4'=40) 7'=40)

('NE66 ('NE71

5'=40) 6'=40)

PSR02 ('SR03 ('SR04 ('SR05 ('SR06 PSR07 ('SR08 ('TD15 PTS01 ('TS03

0'=30) 4'=30) 9'=30) ('SR04 41=30) ('SR05 0'=30) 0'=10) 41=10) ('SR08 2'=40) 4'=20 )('TS01 9'=20) ('TS03

5'=20) 0'=20)

('TS05 ('TS06

7'=20) 9'=20)

(ITS05 ('TS06

81=20) 0'=20)

('TS09 PTS10

6'=20) 4'=20)

('TS09 ('TS10

7'=20) 5'=20)

('TS14 ('TS16 ('TS17 ('TS18 ('TS19

71=20) 0'=20) 9'=20) 4'=20) 01=20)

('TS14 ('TS17 ('TS17 (ITS18

8'=20) 51=20) 0'=20) 51=20)

('TS21 ('TS23 PTS24 ('TS25

4'=10) 3'=20) 0'=20) 4'=20)

('TS23

4'=20)

(ITS25

51=20)

(ITS29 MA06 MA08 (ILA09

61=10) 2=60) 0'=60) 7'=60)

('LA12

0'=60)

31=60)

('LA14

4'=60)

('iA14

5=60)

5'=60) 9'=60) 3'=60)

('LA19 (LA22

5'=60) 0'=60)

3'=10) 2'=20) 9'=20) 3'=20) 0'=20) 51=10) V=60) 9'=60) 6=60) 71=60) 9'=60)

(IY014'=70) ('BL06'=90) ('DG14'=90) ('HA02'=90) ('JE03'=90) ('M28'=90)

('Y021'=70) ('BL08'=90) ('DG16'=90) ('HS01'=90) ('LL29'=90) ('NG201=90)

0'=30) 5'=30)

5'=10)

('Y025'=70) ('BN12'=90) ('DN07'=80) ('HU05'=80) ('LN04'=90) ('NROS'=90)

(IPLO71=90) ('P039'=90) ('RG111=90) ('SE271=90) ('SK17'=90) ('TR18'=90) ('WFOB'=80) (IWF101=80) INTO geogl VARIABLE LABELS geogl 'county'. EXECUTE .

RECODE (ICA01 (ICA02 ('CA03 ('CA04 (ICA05 ('CA06 ('CA07 (ICA08 (ICA10 (ICAll PCA12 ('CA14 (ICA15 (ICA18 ('CA23 PCA28 (IDHOl ('DH02 (IDHO3 (IDHO4 PDH05 ('DHO6 (IDHO7 PDH08 (IDHO9 ('DLO1 PDL02 (IDLO3 ('DL04 ('DLO5 (IDLO6 ('DL07 ("-DL08 ('DL09 ('DL10 ('DL11 PDL12 ('DL13 PDL14 ('DL15 PDL16 PDL17 PNE01 ('NE01 ('NE02 PNE03 ('NE04 PNEOS PNE06 PNE07 PNE09 PNE10 PNEll ('NE13

post 11=52) 4'=52) 8'=52) 8'=52) 11=51) 4'=52) 8=51) 11=52) 11=53) 71=53) 4'=51) 1'=51) 61=51) 11=62) 3'=62) 6'=62) 11=14) V=11) 11=31) 41=35) 8'=35) V=14) 61=14) 5'=13) 6'=13) V=12) V=12) 6'=12) V=16) 4'=16) 11=70) 8'=70) 1'=70) 3'=70) 4'=70) 6'=70) 81=17) V=18) 6'=18) 8'=18) 6'=16) 8'=16) V=32) 6'=32) V=32) 1'=32) 5'=32) 1'=32) V=32) 7'=32) 51=31) 8'=31) 9'=31) 6'=33)

('CA01 ('CA02 ('CA03 PCA04 (CA05 ('CA06 PCA07 PCA08 ('CA10 ('CAll ('CA12 ('CA14 ('CA15 ('CA19 ('CA24 ('CA28 ('DHOI ('DH02 PDH03 PDH04 ('DH05 ('DH06 ('DH07 ('DHOB ('DH09 ('DLO1 ('DL02 ('DL03 ('DL04 ('DLO5 ('DL06 ('DL07 ('DLO8 ('DL09 ('DL10 ('DL11 ('DL12 ('DL13 PDL14 ('DL15 ('DL16 PDL17 ('NE01 ('NE01 ('NE02 ('NE03 ('NE04 ('NE05 ('NE06 ('NE08 ('NE09 ('NE10 ('NE11 ('NE13

2'=52) 5'=52) 9'=52) 9'=52) 2'=51) 5'=52) 9'=51) 2'=52) 2'=53) 8'=53) 5'=51) 2'=51) 7'=51) 1'=62) 3'=62) 7=62) 2'=14) 2'=11) 2'=31) 5'=35) 9'=35) 2'=15) 7'=14) 6'=13) 7'=13) 2'=12) 2'=12) 7'=12) 2'=16) 5'=16) 2'=70) 9'=70) 2'=70) 4'=70) 5'=70) 71=70) 9'=17) ý2'=18) 7'=18) 9'=18) 7'=16) 9'=16) 2'=32) 7=32) 2'=32) 2'=32) 6'=32) 2'=32) 2'=32) V=31) 6'=31) 9'=31) 0'=31) 7'=32)

'CA01 'CA02 'CA03 ICA04 'CAOS 'CA06 'CA07 'CA09 'CA10 'CAll 'CA13 'CA14 'CA15 'CA20 CA25 CA28 'DHOl 'DH02 'DH03 'DH04 'DH05 'DH06 'DH07 'DH08 'DH09 'DLO1 'DL02 'DL03

3'=52) 6'=52) 0'=52) 01=52) 3'=51) 6'=52) 0'=51) 3'=53) 3'=53) 9'=53) 9'=51) 3'=51) 8'=51) 11=62) 5'=62) 8'=62) 3'=14) 3'=11) 31=11) 6'=14) 01=35) 3'=15) 8'=14) 7'=13) 8'=13) 3'=12) 3'=17) 8'=12)

'DL05 'DL06 'DL07 'DL08

6'=16) 3'=70) 0'=70) 3'=70)

'DL10

('RG12'=90) ('TW02'=90)

( 'S65'=80) ('WF03'=80)

('CA02

7'=52)

('CA05 ('CA05 ('CA06

6'=52) 4'=51) 7'=52)

('CAll ('CA13 ('CA14 ('CA16 (ICA21 ('CA26 ('CA28 (IDH01

01=53) 0'=51) 4'=51) 61=53) 21=62) 3'=62) 9'=62) 4'=14)

('CA02

( 'S73'=80) ('WF05'=80)

8'=52)

PCA05 (ICA05

7'=52) 51=51)

(ICA14 (ICA17 ('CA22 ('CA27

5'=Sl) 41=53) 2'=62) 0'=62)

(IDHOl

5'=14)

(IDHO3 4'=11) (IDHO4 71=35) (IDHO8 01=13) ('DH06 41=14) ('DH07 9'=13) ('DHO8 8'=13) ('DH09 9'=13) ('DLOI 4'=12)

(IDHO6 ('DH07 ('DHOB ('DH09 ('DLO1

5'=14) 0'=13) 9'=13) 0'=13) 5'=12)

('DL03

9'=12)

('DL03

0'=12)

('DLOS

7'=16)

PDL08

4'=60)

('DLO8

5'=60)

6'=70)

('DL10

7'=70)

'DL12 'DL13 'DL14 'DL15

0'=17) 3'=18) 8'=18) 0'=18)

('DL13 ('DL14

4'=18) 9'=18)

('DL13 ('DL14

5'=17) 0'=18)

'DL17 'NE01 'NE01 'NE02 'NE03 'NE04 'NE05 'NE06 'NE08 'NE09 'NE10 'NE12 'NE13

0'=16) 3'=32) 81=32) 3'=32) 3'=32) 7'=32) 3'=32) 3'=32) 2'=31) 7'=31) 0'=31) 8'=33) 8'=32)

('NE01

4'=32)

('NE01

5'=32)

PNE02 ('NE03 ('NE04 ('NE05 ('NE06 ('NE08

4'=32) 4'=32) 8'=32) 4'=32) 4'=32) 3'=31)

('NE03 ('NE04 ('NE05 ('NE06 ('NE08

5'=32) 9'=32) 5'=32) 5'=32) 4'=31)

('NE12

9'=33)

('NE12

0'=33)

('NE15 6'=32) ('NE16 3'=31) (INE17 7'=31) ('NE20 9'=44) ý'NE21 V=31) ý'NE22 5'=46) ý'NE23 6'=43) ý'NE24 V=43) 'NE25 8'=33) : 'NE26 V=33) 'NE28 6'=33) 'NE29 6'=33) 'NE30 V=33) 'NE31 V=34) 'NE32 3'=34) 'NE33 V=34) 'NE34 6'=34) 'NE35 9'=34) 'NE37 1'=35) 'NE38 '7'=35) 'NE39 V=31) 'NE41 8'=45) 'NE44 6'=45) 'NE46 V=45) 'NE47 5'=45) 'NE47 0'=45) 'NE49 9'=45) 'NE61 V=44) 'NE61 6'=44) 'NE63 8'=46) 'NE65 7'=44) 'NE66 V=41) 'NE67 5'=41) 'SR01 V=35) 'SR02 7'=35) 'SR03 V=35) ISR04 61=35) 'SR05 V=35) 'SR06 7'=34) 'SR07 V=15) 'SROS V=15) 'TD11'=90) 'TS01 V=22) 'TS03 6'=22) 'TS04 2'=22) 'TS05 4'=22) 'TS06 6'=23) 'TS07 8'=22) 'TS08 9'=22) 'TS10 V=23) 'TS11 6'=23) 'TS12 V=23) 'TS13 4'=23) 'TS15 9'=24) 'TS17 6'=24) 'TS18 V=24) 'TS19 7'=24) 'TS20 V=24) 'TS21 V=12) 'TS22 5'=24) 'TS24 V=21) 'TS25 V=21) 'TS26 8'=21) 'TS27 3'=21) 'LA05 9'=63) 'IA07 7'=63) 'LA09 4'=63) 'LA10 5'=63) 'LA12 V=63) 'LA13 9'=61)

PNE15 ('NE16 ('NE18 ('NE20 ('NE21 ('NE22 PNE23 ('NE24 ('NE25 ('NE26 ('NE28 ('NE29 (INE30 ('NE31 ('NE32 (INE33 (INE34 ('NE36 ('NE37 ('NE38 ('NE39 ('NE42 ('NE45 PNE46 PNE47 ('NE48 (INE49 ('NE61 CNE62 (INE63 ('NE65 (INE66 (INE68 (ISR01 (ISR02 ('SR03 (ISR04 ('SR05 ('SR06 ('SR07 ('SROB ('TD12 ('TS01 PTS03 ('TS04 ('TS05 ('TS06 ('TS07 ('TSOB ('TS10 ('TS11 ('TS12 ('TS13 ('TS15 ('TS17 ('TS18 ('TS19 PTS20 ('TS21 ('TS23 ('TS24 ('TS25 ('TS26 ('TS27 ('LA05 ('LAOS ('LA09 (LA11 ('LA12 ('LA13

7=32) 41=31) 0'=44) 0'=44) 5'=31) 6'=46) 7'=33) 2'=43) 91=33) 2'=33) 7'=33) V=33) 21=33) 2'=34) 4'=34) 2'=34) V=34) 0'=34) 2'=35) 81=35) 2'=31) 5'=45) 5'=45) 2'=45) 6'=45) V=45) 0'=45) 2'=44) 5'=46) 9'=46) 8'=44) 21=41) 7'=41) 2'=35) 81=35) 2'=35) V=35) 2'=35) 8'=35) 8'=15) 2'=15) 4'=42) 21=22) V=22) 3'=22) 5'=22) 7'=23) 9'=22) 0'=22) 2'=23) V=23) 2'=23) 5'=23) 0'=24) 7'=24) 2'=24) 8'=24) 2'=24) 2'=16) V=24) 8'=21) 2'=21) 9'=Zl) V=15) 01=63) 8'=63) 5=63) 6'=63) 8'=63) 0'=61)

PNE15 PNE16 ('NE19

8'=32) 5'=31) V=45)

PNE21 ('NE22 (NE23 PNE24 ('NE25 CNE26 ('NE28 ('NE29 PNE30

6'=31) 7'=46) 8'=43) 3'=43) 0'=43) 3'=33) 8'=33) 8'=33) 3'=33)

('NE32 ('NE33 ('NE34

(INE15 PNE16 'NE19

91=32) 6'=13) 2'=45)

'NE23 'NE24

9'=43) 4'=43)

'NE26 'NE28 'NE29 'NE30

5'=34) 3'=34) 8'=34)

PNE37 ('NE38 ('NE40 CNE42

(NE15

0'=32)

PNE24

5'=43)

4'=43) 9'=33) 9'=33) 4'=33)

PNE27 PNE28 PNE29

0'=33) 0'=33) 0'=33)

'NE33 'NE34

4'=34) 9=34)

('NE33 ('NE34

5'=34) 01=34)

3'=35) 9'=35) 3'=31) 61=45)

'NE38 'NE40 'NE43

0'=35) 4'=31) 7'=45)

('NE46 ('NE47 ('NE48

3'=45) V=45) 2'=45)

'NE46 'NE47 'NE48

4'=45) 8'=45) 3'=45)

('NE47 ('NE48

9'=45) 41=45)

('NE61

3'=44)

'NE61

4=44)

('NE61

5'=44)

('NE63 ('NE65 ('NE66 ('NE69 ('SR01 ('SR02 ('SR03 ('SR04 ('SR05 ('SR06 ('SR07 ('SROB ('TD15 ('TS01 ('TS03 ('TS02 ('TS05 ('TS06 ('TS07 ('TS09 ('TS10 ('TS11 ('TS12 ('TS14 ('TS16 ('TS17 ('TS18 ('TS19

0'=46) 9'=44) 31=41) V=41) 3'=35) 9'=35) 3'=35) 8'=35) 31=35) 9'=35) 9'=15) 3'=15) V=42) 3'=22) 8'=22) V=22) 6'=22) 8'=23) 0'=22) 5'=23) 3'=23) 8'=23) V=23) 6'=23) 9'=24) 8'=24) 3'=24) 9'=24)

'NE64 'NE65 'NE66 'NE70

6'=46) 0'=44) 4'=41) 7'=42)

(INE66 ('NE71

5'=42) 6'=42)

'SR02 'SR03 'SR04 'SR05 'SR06 'SR07 'SROS 'TD15 'TS01 'TS03

0'=3ý) 4'=35) 9'=35) 4'=35) 0'=35) 0'=15) 4'=15) 2'=42) 4'=22 9'=22)

('SR04 PSR05

0'=35) 5'=35)

('SROS

5'=15)

'TS05 ITS06

('TS21 ('TS23 ('TS24 ('TS25 ('TS26 ('TS28 (ILA06 (ILA08 ('LA09 (ILA11 ('LA12

3'=16) 2'=24) 9'=21) V=21) 0'=21) 5'=15) V=62) 9'=63) 6'=63) 71=63) 9'=63)

)('TS01 (ITS03

5'=22) 0'=22)

7'=22) 91=23)

(ITS05 ('TS06

8'=22) 0'=23)

'TS09 'TS10

6'=23) 4'=23)

(ITS09 ('TS10

7'=23) 51=23)

'TS14 ITS16 'TS17 'TSIS 'TS19

7'=23) 01=24) 9'=24) 4'=24) 0'=24)

('TS14 ('TS17 (ITS17 (ITSIS

8=23) 5'=24) 01=24) 5'=24)

'TS21 'TS23 'TS24 'TS25

4'=16) 3'=24) 0'=21) 4'=21)

('TS23

4'=24)

(ITS25

5'=21)

'TS29 'LA06 'LA08 , IA09

6'=16) 2=63) 0'=63) 7'=63)

'LA12

0'=63)

PLA14 1'=61) ('LA14 2'=61) MA14 3'=61) PLA14 4 '=61) MA14 PLA15 8=61) PLA16 7'=61) MA17 71=63) PLA18 4'=62) PLA18 51= 62) PLA19 5 '=62) MA20 6'=63) PLA21 8'=63) PLA22 9'= 63) PLA22 0 '=63) PLA23 1'=63) PLA23 2'=63) MA23 3'= 63) ('YO03'=70) ('YO04'=70) ( 'YO13'=70) ('YO141=70) ('Y021'=70) PA108 1=90) ( 'B26'=90) ( 'BD19'=90) ('BL061=90) PBL081=90) PDA12 ' =90) ( DD10'=90) ( 'DG02'=90) ('DG141=90) ('DG16'=90) ( ' DN17 1=90) ( 'GL14'=90) ( 'GU14'=90) ('HA02'=90) PHS01'=90) ( ' HU07 ' =80) ( 'HU17'=80) ( 'HX02'=80) ('JE03'=90) ('LL29'=90) ( ' LS08 1=80) ( 'LS12'=80) ( 'LS17'=80) (IM281=90) (ING201=90) ( ' PL07 1=90) ( 'P039'=90) ( 'RG11'=90) ('RG12'=90) PS65'=80) PSE27 1=90) ( 'SK171=90) ( 'TR18'=90) ('TW02'=90) ('WF03'=80) ( ' WF08 ' =80) ( IWF10'=80) INTO geog2 VARIABLE LABELS geog2 'district'. EXECUTE .

5'=61)

('Y025'=70) ('BN12'=90) ('DN07'=80) ('HU05'=80) ('LN04'=90) ('NR05'=90) ('S73'=80) ('WF05'=80)

"PHD-A-LL. SPS = SYNTAX FILE FOR ESD / SER / TITLE for 'as SUBTITLE age, of 01.01.99 - calculations

PSR / ' etc.

DNP /

COMPUTE , xdate. , xdate. EXECUTE

xdate. agelO month(datelO) year(datelO), = YRMODA(xdate. / 365.25 year(dob), xdate. mday(datelO)) - YRMODA(xdate. / 365.25 mday(dob))

COMPUTE

time

, xdate. , xdate. EXECUTE

month(dob)

. year(datell), xdate. = YRMODA(xdate. 365.25 YRMODA(xdate. mday(datell)) 365.25 mday(datelO))

RECODE time 3.49=2) (2.50 (3.50 4.00=1) thru (3.00 thru (1.00 2.49=4) (1.50 1.99=5) thru thru thru 0.99=7) (0.00 0.49=8) thru INTO recode2 Irecode2'. VARIABLE LABELS recode2 EXECUTE .

month(datell) year(datelO),

xdate.

365.25 / 365.25

COMPUTE agel = YRMODA(98,13,00) xdate. month(dob), xdate. mday(dob)) EXECUTE .

YRMODA(xdate.

month(dob)

year(dob),

COMPUTE , xdate. , xdate. EXECUTE

xdate. month(date25) year(date25), age25 = YRMODA(xdate. / 365.25 YRMODA(xdate. xdate. year(dob), mday(date25)) / 365.25 mday(dob))

COMPUTE EXECUTE

diff5

COMPUTE EXECUTE

age2

COMPUTE EXECUTE

age3

COMPUTE EXECUTE

diffl

COMPUTE EXECUTE

diff2

COMPUTE EXECUTE

diff6

COMPUTE EXECUTE

diff4

COMPUTE , xdate. , xdate. EXECUTE

xdate. month(date9l) year(date9l), age9l = YRMODA(xdate. / 365.25 xdate. year(dob), mday(date9l)) - YRMODA(xdate. / 365.25 mday(dob))

COMPUTE , xdate. , xdate. EXECUTE

xdate. month(date92) year(date92), age92 = YRMODA(xdate. / 365.25 xdate. year(dob), mday(date92)) - YRMODA(xdate. / 365.25 mday(dob))

COMPUTE

month(date93) xdate. year(date93), age93 = YRMODA(xdate. / 365.25 year(dob), xdate. mday(date93)) - YRMODA(xdate. / 365.25 mday(dob)) .

age25

month(datelO)

(2.00 thru

2.99=3) thru (0.50 1.49=6)

COMPUTE age20 xdate. month(date2O) year(date2O), = YRMODA(xdate. / 365.25 YRMODA(xdate. xdate. year(dob), xdate. mday(date20)) , / 365.25 xdate. mday(dob)) , EXECUTE .

, xdate. , xdate.

PHD

month(dob)

age20

-

. + 1900

onsetl

xdate.

year(dob)

. diag

+ 1900

-

xdate.

year(dob)

. = age3

-

age2

. = onsetl

-

onset2

. = diag

-

told

. = diffl-

diff3

. month(dob)

. month(dob)

. month(dob)

EXECUTE

.

COMPUTE EXECUTE

diff7

COMPUTE , xdate. , xdate. EXECUTE

xdate. year(date98), month(date98) age98 = YRMODA(xdate. / 365.25 xdate. year(dob), mday(date98)) - YRMODA(xdate. / 365.25 mday(dob))

= age93

age9l

-

.

.

COMPUTE time3 year(date98), xdate. month(date98) = YRMODA(xdate. 365.25 YRMODA(xdate. year(datelO), xdate. mday(date98)) , 365.25 xdate. mday(datelO)) , EXECUTE RECODE time3 (6.00 (4.50 (3.00 (1.50 (0.00 VARIABLE EXECUTE

month(dob)

5.9999=2) (5.50 thru 4.4999=5) (4.00 thru 2.9999=8) (2.50 thru (1.00 1.4999=11) thru (-38.0 thru -0.0999=14) lzecodell.

6.4999=1) thru 4.9999=4) thru 3.4999=7) thru 1.9999=10) thru 0.4999=13) thru LABELS recodel

xdate.

month(datelO)

(5.00 5.4999=3) thru (3.50 3.9999=6) thru (2.00 2.4999=9) thru 0.9999=12) (0.50 thru INTO recodel

.

"PHD-DEC. SPS = SYNTAX FILE TITLE 'as SUBTITLE of 01.05.98 - decimal COMPUTE EXECUTE

age10

COMPUTE EXECUTE

time

COMPUTE EXECUTE

age20

COMPUTE EXECUTE

agel

COMPUTE EXECUTE

age25

COMPUTE EXECUTE

diff5

COMPUTE EXECUTE

age9l

COMPUTE EXECUTE

age92

COMPUTE EXECUTE

age93

COMPUTE EXECUTE

diff7

COMPUTE EXECUTE

age98

COMPUTE EXECUTE

time3

+ 0.5)

(age10

-

FOR ESD places'

(MOD((agelO

/

SER /

PSR

+ 0.5),

l))

DNP /

PHD

. (((time

*

(((age20

+ 0.5)

10)

*

10)

-

+ 0.5)

(MOD(((time

*

10)

+ 0.5),

l)))/10

*

10)

+ 0.5),

l)))/10

-

(MOD(((age25

*

10)

+ 0.5),

l)))/10

-

(MOD(((diff5

*

10)

+ 0.5),

l)))/10

*

10)

+ 0.5),

l)))/10

-

(MOD(((age20

. (agel

+ 0.5)

-

+ 0.5),

(MOD((agel

l))

. (((age25

*

10)

+ 0.5)

(((diff5

*

10)

+ 0-5)

(((age9l

*

10)

+ 0-5)

-

(MOD(((age9l

(((age92

*

10)

+ 0.5)

-

(MOD(((age92

*

10)

+ 0.5),

l)))/10

(((age93

*

10)

+ 0.5)

-

(MOD(((age93

*

10)

+ 0.5),

l)))/10

(((diff7

*

10)

+ 0.5)

-

(MOD(((diff7

*

10)

+ 0.5),

l)))/10

(((age98

*

10)

+ 0.5)

-

(MOD(((age9B

*

10)

+ 0.5),

l)))/10

(((time3

*

10)

+ 0.5)

-

(MOD(((time3

*

10)

+ 0.5),

l)))/10

.

.

.

.

.

.

.

.

"PHD-SF36. TITLE 'SF36 SUBTITLE COMPUTE EXECUTE

FOR ESD / SPS = SYNTAX FILE COMPUTATIONS & EuroQol as of

ostatel=anxl

+

(10*discl)

+

SER / PSR / 06.07.971

(100*actl)

+

DNP /

(1000*selfl)

PHD

+

(10000*mobl

.

COMPUTE Ml=9. (mobl eq I)ml=O. if if (mobl eq 2)ml=0.0665. if (mobl eq 3)ml=(0.0665*2)+0.0678. COMPUTE sl=9. if (selfl eq 1)sl=O. (selfl if eq 2)sl=0.0834. if (selfl eq 3)sl=0.0834*2. COMPUTE al=9. (actl if eq 1)al=O. if (actl eq 2)al=0.0953. if (actl eq 3)al=0.0953*2. COMPUTE dl=9. if (discl eq 1)dl=O. if (discl eq 2)dl=0.0522. if (discl eq 3)dl=(0.0522*2)+0.1369. COMPUTE xl=9. if (anxl eq 1)xl=O. if (anxl eq 2)xl=0.0629. if (anxl eq 3)xl=(0.0629*2)+0.0825. (9). missing values ral sl al dl xl COMPUTE eurol=((l-(ml+sl+al+dl+xl+0.1716))*100). FORMAT eurol(F5.2) . if (mobl eq 1& selfl eq 1& actl eq 1& eurol=100.00 EXECUTE COMPUTE ostate2=anx2 EXECUTE .

+ (10*disc2)

+ (100*act2)

COMPUTE m2=9. if (mob2 eq 1)m2=0. if (mob2 eq 2)m2=0.0665. if (mob2 eq 3)m2=(0.0665*2)+0.0678. COMPUTE s2=9. if (self2 eq 1)s2=0. if (self2 eq 2)s2=0.0834. if (self2 eq 3)s2=0.0834*2. COMPUTE a2=9. if (act2 eq 1)a2=0. if (act2 eq 2)a2=0.0953. if (act2 eq 3)a2=0.0953*2. COMPUTE d2=9. if (disc2 eq 1)d2=0. if (disc2 eq 2)d2=0.0522. if (disc2 eq 3)d2=(0.0522*2)+0.1369. COMPUTE x2=9. if (anx2 eq 1)x2=0. if (anx2 eq 2)x2=0.0629. if (anx2 eq 3)x2=(0.0629*2)+0.0825. missing values m2 s2 a2 d2 x2 (9). COMPUTE euro2=((l-(m2+s2+a2+d2+x2+0.1716))*100). FORMAT euro2(F5.2) . if (mob2 eq 1 & act2 eq 1& eq self2 euro2=100.00 EXECUTE . COMPUTE pfl=(((vigl + modl +walcl + bathl)-10)/20)*100. EXECUTE .

+ liftl

COMPUTE pf2=(((vig2 + mod2 + lift2 + walc2 + bath2)-10)/20)*100.

discl

disc2

eq 1&

eq 1)

anxl

+ (1000*self2)

eq 1

anx2

+ (10000*mob2

eq 1)

+ sevl

+ onel

+ bendl

+ walkl

+ walbl

+ sev2

+ one2

+ bend2

+ walk2

+ walb2

EXECUTE

. + accl

+ liml

+ hadl)-4)/4)*100.

+ acc2

+ lim2

+ had2)-4)/4)*100.

COMPUTE EXECUTE

rpl=(((cuti

COMPUTE EXECUTE

rp2=(((cut2

COMPUTE EXECUTE

bpl=(((howl

COMPUTE EXECUTE

bp2=(((how2

COMPUTE EXECUTE

ghl=(((genl

COMPUTE EXECUTE

gh2=(((gen2+

COMPUTE EXECUTE

vtl=(((lifel

COMPUTE EXECUTE

vt2=(((life2

COMPUTE EXECUTE

sfl=(((extl

+ socl)-2)/8)*100.

COMPUTE EXECUTE

sf2=(((ext2

+ soc2)-2)/8)*100.

COMPUTE EXECUTE

rel=(((timel

COMPUTE EXECUTE

re2=(((time2

COMPUTE EXECUTE

mhl=(((nervl

COMPUTE EXECUTE

mh2=(((nerv2

.

. + painl)-2)/9)*100.

. + pain2)-2)/9)*100.

. + illl

+ anyl

+ excl)-5)/20)*100.

+ worl

. i112

+ any2

+ wor2

+ exc2)-5)/20)*100.

. tirl)-4)/20)*100.

+ lotl

+ wornl

+

+ lot2

+ worn2

+ tir2)-4)/20)*100.

. '

.

. + lessl

+ workl)-3)/3)*100.

+ less2

+ work2)-3)/3)*100.

+ downl

+ calml

+ lowl

+ happl)-5)/25)*100.

+ down2

+ calm2

+ low2

+ happ2)-5)/25)*100.

.

.

.

.

FOR ESD 04-07-97'

/

SER /

PSR /

"PHD-QUAL. TITLE SPS= 'qualitative SUBTITLE

SYNTAX FILE data as of

C6MPUTE quall EXECUTE .

= partl

+ peopl

+ burdl

+ embl

COMPUTE qual2 EXECUTE .

= part2

+ peop2

+ burd2

+ emb2 + app2

COMPUTE qual3 EXECUTE .

= quall

- qual2

+ appl

DNP /

PHD

+ conl

+ copl

+ supl

+ con2

+ cop2

+ sup2

"PHD-PSR. TITLE FOR PSR, DNP and SPS = SYNTAX FILE SUBTITLE 'for as of 23.06.97 re Marjan' analysis COMPUTE fdqall fdq9 + fdqlO fdql8 + fdql9 fdq27 EXECUTE

PHDI'

+ fdq2 + = fdql + fdql2 + fdqll + fdq20 + fdq2l

fdq3 + fdq5 + fdq7 + fdq4 + fdq6 + fdql6 + fdql3 + fdql4 + fdql5 + fdq25 + fdq22 + fdq23 + fdq24

= fdq5

+

fdq9

= fdq2

+ fdq4

COMPUTE EXECUTE

fdqsoc

COMPUTE EXECUTE

fdqphy

COMPUTE EXECUTE

fdqsel

=

COMPUTE EXECUTE

fdqlei

= fdq3

COMPUTE EXECUTE

fdqoth

COMPUTE bcs9 bcs18 EXECUTE

bcsall bcslO + bcs19

COMPUTE EXECUTE

bcsspe

COMPUTE EXECUTE

bcspos

COMPUTE EXECUTE

bcseva

COMPUTE EXECUTE

bcsten

fdq7

+

fdqs + fdql7 + fdq26 +

fdq22

fdql6

+ fdql7

+ fdq20

+

+ fdql2

+ fdql3

+ fdql9

+ fdq27

+

+

+ + +

fdq24

. + fdq8

. fdql

+ fdqlO

+ fdq6

+ fdql5

+

+ fdq25

fdq2l

+

fdqll

. =

fdql4

+

fdql8

+ fdq23

+

fdq26

. + bcs5 + bcs6 + bcs7 + bcs4 + bcs3 + bcs2 = bcsl + bcs16 + bcs14 + bcs15 + bcs13 + bcsll + bcs12 + bcs22 + bcs20 + bcs2l

+ bcs8 + bcs17

+ +

. = bcs3

+ bcs7

+ bcslO

= bcsl

+ bcs6

+ bcs8

= bcs2

+ bcs5

+ bcs14

= bcs4

+ bcsll

+ bcs13

+ bcs15

+ bcs2l

+ bcs22

. + bcs9

+ bcs16

+ bcs17

+ bcs20

. + bcs19

.

.

COMPUTE bditot bdi9 + bdilO bdi18 + bdil9 EXECUTE . COMPUTE sestot + seslO ses9 EXECUTE COMPUTE EXECUTE

pesteem

COMPUTE EXECUTE

nesteem

COMPUTE EXECUTE

iodtot

COMPUTE csilo EXECUTE

csitot + csill

+ bdi5 + bdi6 + bdi4 + bdi7 + bdi2 + bdi3 = bdil + bdil4 + bdil5 + bdil6 + bdil3 + bdil2 + bdill + bdi22 + bdi2l

sesl

+ ses2

+ ses3

+

ses4

+ ses5

sesl

+ ses3

+ ses4

+ ses7

+ seslO

ses2

+ ses5

+ ses6

+ sesS

+ ses9

+ ses6

+ ses7

+ bdi8 + bdil7

+ +

+

+ ses8

.

+ iod4

+ iod5

+ iod6

+ iod7

+ csiS + csi4 + csi3 csi2 + csil4 + csil2 + csil3

+ csi6

+ csi7

+ csi8

iodl

+ iod2

+ iod3

.

.

+ csi9

+

"PHD-ENV. TITLE SPS = SYNTAX FILE 'as SUBTITLE of 09.12.96 - Q're

IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF IF

(env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env. (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env (env

EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ

1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1)

envaa envab envac envad envae envaf envag envah envai envaj envak enval envam envan envao envap envaq envar envas envat envau envav envaw envax envay envaz envba envbb envbc envbd envbe envbf envbg envbh envbi envbj envbk envbl envbm envbn envbo envbp envbq envbr envbs envbt envbu envbv envbw envbx envby envbz envca envcb envcd envcc enved envce envcf envcg envch envci envcj envck envcl envcm

= = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = =

0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0.

FOR ESD 80 : (IF

/

SER / PSR / DNP env = 1) formulae

PHD

IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env IF (env EXECUTE

EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ

envcn envco envcp envcq envcr envcs envct envcu envcv envcw envcx envcy envcz envda envdb envdc envdd envde envdf envdg envdh envdi envdj envdk envdl envdm envdn envdo envdp envdq envdr envds envdt envdu envdv envdw envdx envdy envdz

= = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = = =

0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0. 0.

. 'as

SUBTITLE IF (alg IF (alg IF (alg IF (alg IF (alg IF (alg IF (alg IF (alg IF (alg IF (alg IF (alg IF (alg IF (alg IF (alg EXECUTE

1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1) 1)

EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ EQ .

2) 2) 2) 2) 2) 2) 2) 2) 2) 2) 2) 2) 2) 2)

of

08.07.98

alga algb algc algd alge algf algg algh algi algj algk algl algm algn

= = = = = = = = = = = = = =

2. 2. 2. 2. 2. 2. 2. 2. 2. 2. 2. 2. 2. 2.

- Q're

80

:

(IF

alg

= 2)

formulae

I

The Social and EconomicImplicationsof SpasmodicTortioollis in the Notth Eastof England

APPENDIX E OTHER DOCUMENTATION The following documentsare enclosed. Letter from the Doctor in the Cost Utility Analysisinviting patients to participate. 2. The ConsentForm - completedby the patient 3. The Clinical Information Form - completedby the researcherfor the Epidemiology 4. The Clinical AnalysisForm - completedafter the Cost Utility Analysis

15ý

DYSTONIA. S.,

BOTULINUM

TOXIN

AND QUALITY

OF LIFE

illnesses Dystonias are rather unusual and our understanding of is it is instance, them far from known For not complete. how common they exactly are, what sort of people are affected their lives. or how having a dystonia alters it the has also In past proved in the thankfully last although injections found Botulinum Toxin to be of great help. muscles

dystonia, to difficult treat have few years many patients into the overactive or, around

Because we are attempting of these uncertainties, the people the who attend with a dystonia .............. injections the effect Toxin examine of the Botulinum quality of life.

to

study all and to upon their

in our study, be asked to If you choose to participate you will These would complete ask questions a series of questionnaires. to measure how you feel the effectiveness about of and attempt the treatment. The questionnaire would need to be completed just before once again when the injections a set of injections, when the next are fully again set of injections and then active be the due. total, to In are you will asked complete the next questionnaire every six weeks for six to approximately be sent to The questionnaire you in the nine would months. take post will no with a stamped addressed envelope and it to complete. longer than fifteen minutes into like the study, to ask In addition, we would upon entering what you sort of person are. some detailed about questions interview informal be done by means of a short This with would for the Dystonia Society, Mr Ginger Butler, which a researcher is is a national to people to provide support whose aim charity by dystonia. affected information by the the All will remain gathered study treatment you are given absolutely and the medical confidential involved in it. identical be or not you will whether are in the the be published the Hopefully study will of results in involved the in to literature guide medical order others displayed however they be treatment dystonia, would as of identity the the any of statistics reveal of and not individuals involved. in this be I would to take Would part study? you be willing if form the your giving grateful complete enclosed you could it if decision you on your next appointment. with and bring both Mr Butler further the matter like to discuss and you would be happy to oblige I would when I next see you. on hospital

notepaper,

signed

by

the

doctor

treating

the

patient

DYSTONIAS,

I OF

BOTULINUN

...............................

(ADDRESS)

..............................

(Post

SIGNED DATE

*

PLEASE

/

Code)

(Telephone

.............................. AGREE

OF LIFE

(NAME)

..............................

*

AND QUALITY

TOXIN

DO NOT AGREE

TO TAKE

PART

IN

THIS

No) STUDY.

.......................... ..........................

DELETE

WHAT DOES NOT APPLY

TO YOU

PLEASE REMEMBER TO BRING THIS FORM WITH YOU WHEN YOU NEXT ATTEND THE CLINIC FOR YOUR BOTULINUM TOXIN INJECTIONS AND EITHER GIVE IT TO YOUR DOCTOR OR ASK TO SEE MR BUTLER WHO WILL BE ABLE TO ANSWER ANY OF YOUR QUESTIONS ABOUT THE STUDY.

Epidemiological CLINICAL

(ESD)

Survey of Dystonia INFORMATION FORM

information Please the following for complete new patient every who is the minimum throughout NB : This 1996. arrives at your clinic information is for the Epidemiology to essential required and it have true that validity all new patients and accuracy are logged. Please forward Dystonia to Hunters A. G. Butler, Research, Moor Regional Rehabilitation Hunters Road, Newcastle. NE2 4NR. Centre, ESD Number

:

Hospital

:

No

Patient's DoB

............

Name

:

:

by

:

Status

Marital

Gender

.................................

No

...........................................

Tel

...........................................

Post

What

is

the

Date

of

Onset

Details

Details dosage

Any

of

patient's

any

:

exact

other

Clinical

Co-morbidity

comments

Diagnosis

Date

..............

of any known and frequency

team)

research

.................

............

Address

be completed

Hospital

............

Full

:

(to

(if

of

known)

Diagnosis

include

relating medications is not essential (this

you

would

wish

to

:

make,

to

:

.............

:

.............

Code

............

..................

..................

onset

dates

if

dystonia their information)

eg Dystonia

Society

poss.

include

info

CLINICAL Survey

Number

First

Interview

Dates

of

01 02 03 04 05 06 07 08 09 10 11 Exact

Hospital

...... :

ANALYSIS No

Final

.............

Questionnaires

:

Injection

FORN Hospital

......... interview Dates

:

: NRI/HMH .............

Dosage

Number

...................

...............

......

......

...................

...............

......

......

...................

...............

......

......

...................

...............

......

......

...................

...............

......

......

...................

..............

......

......

...................

...............

......

......

...................

...............

......

......

...................

...............

......

......

...................

...............

......

......

...................

...............

......

......

Clinical

Diagnosis

&

....................................

............................................................... Details

of

any

Co-morbidity

:

.................................

............................................................... Drug

Name

Dosage

Frequency

Cost

per

dose

Total

cost

Dystonia -A comprehensiveand Iongtitudinal study in the North East of England

APPENDIX F VERBATIM ANSWERS The following are the verbatim answersto the question: "Describe ill detail (in terms of hand used,area of head, neck orface touched), the gesture that you use(d) to keep your head straight. Perform this gesture in front of a mirror before answering and write your answersoil a separatepiece ofpaper. " The numbersin the brackets,eg. (016), relate to the numberof the anonymous patient. (016) luse thetips ofthe fingers of my left hand to the left side of my chin. Ionlyuse lightpressure. My head starts to move a little before the fingers touch my chin. Iam unable to bend or tilt my neck to the left now. I just turn my whole torso to compensate.My right shoulderis higher than my left and I do not stand as straight as I usedto, (026) On different occasionsI would use different geste's,ie., when driving etc., I would have to put my left hand around the back of my neck and kept a continuous strong pull. When socialising,I would smoke cigarettesas the right hand movementto my mouth would help for short periods. I would move my left index finger side to the centre of my chin and lightly touch. None of thesegeste'sare now neededdue to the Botul-inumToxin injections, although this period hasnot beenasgood as in the past. (040) By placing my handsbehind my neck and pulling neck a little forward. (046) Left handon left side of face. (054) 1 no longer need to do this as my neck has much improved with the Botulinurn Toxin injections. (057) Depending on the situation ; back of head when driving and crossing road and trying to look left ; fight hand on chin when trying to sit still and also when trying to look at someoneon the left side of me. (061) Touch left side of chin with the back of the fingers of the right hand. (072) Put my right hand againstmy chin and try to pushmy headback. (073) 1 sometimeswalk holding my cl-ýnwith my right hand. (075) Hand to mouth, (077) Using the fingers of right hand on the chin to pushthe headto the front. 0

164

Dystonia -A comprehensiveand longtitudinal study in the North East of England

(082) 1 put my left hand up to my neck and hold it from my jaw line and as far back as my hand will go and push hard and then hold for a couple of secondsand then stop, otherwiseit startspulling over harder and then I find it hard to stop holding my neck. (083) 1 used to, before injections, put my left hand down the left side of my face and push hard to keep my headstraight. (088) Right handto rig side of face or drinking (sameside). Cht (090) 1 feel it's like a heavy weight on my left shoulder, so by lifting my left arm up towards my face, I know my head will turn to the centre and into a straight line with my body. It turns before I touch my face, in fact just thinking about my hand touching ) my face seemsto work. (PS :I hope this makessense. (092) Using right or left hand, raise index finger to mid point of chin, touching very lightly (any pushing causesfighting against it). This gesture moved my head back to normal mid-body position for very short periods. I used this method to enableme to relax in bed and so go to sleep. (097) 1 have often tried to keep my head straight with my handbut it never works. (109) Right handto right side of face, (117) When I tried to keep my headstraight I used to use my left hand to hold my face at the front and push hard to keep my head straight. My head goes towards the midline when my handpusheshard. My headwas normaflypulling towards the left. (122) 1 sit or stand with my left hand on the left side of my face or chin and push my heador neck towards the right. (124) Touching left side of chin with left hand. (142) Left handon left side of face. (188) When walking I am almost constantly blowing my nose. This helps to keep my headstraight. When in a sitting position, I cradle my headwith my right hand. (207) When sitting upright, if I can lean my elbow on a table I can keep my head still. When sitting upright my headtrembles slightly at the moment. Lcan press with thumb or third fin90 er half way up my neck and it will stop the shakina for a little while or pressagainstthe cheekbone near the ear on the right side. When laying in bed, I have the most trouble. I can lay on my right side - no trouble. I can lay on my left side when I have beenin bed for half an hour or so. On my back, I always have to put my arm over my headto hold it in place. (209) In addition to touching, when bad, I had to sit so my head was against a wall or similar (this lady is now in remission).

165

Dystonia -A comprehensiveand longtitudinal study in the North East of England

(221) This patient'sanswerswere defined accordingto body part :1. Right hand: a) Touching, brushingor resting in the right side of the face,jaw rear b) Brushing foreheadand / or hair (pulls it back) c) Restingforeheadin head d) Almost any movementacrossupper part of body e) Restingwalking stick or shoppingbag over right shoulder. 2. Left hand: a) Similar to above,but less effective except when handis applied to left side of face b) If I wish to look to the right, eg when about to cross a road or turn towards someone,I tend to force my head around by cupping my chin in my left hand and pushingfairly hard (henceI no longer drive a car). 3. Both hands: Claspedbehind my head, especiallywhen seated Body position: a) If walking with someoneor speakingto someoneI always attempt to stand to their right evento the extent of talking to them acrossmy left shoulderas far as possible b) By standing'militarily'upright somerelief is obtained c) Deliberate'floppy' relaxation of all muscleshelpstemporarily (230) At present, maybe as a result of BT injections, I can turn my head to centre without using my hand. However, in the past, I've used my right hand on my jaw and pushedgently to the centre. (252) 1 use my right hand to cup my chin between my thumb and first fingers and support under my chin with my other fingers. This enablesme to keep my face frontal, so I can seewhere I am walking, but I still don't walk correctly, I seemto sway. (256) 1 hold the left side of my face and push with my left hand to put my face straight. I touch my left cheek. I put both my handson my neck to bring my neck forwards. (257) 1 usedto hold my chin or side of my face with my right hand for approximately2 years. I now occasionallyhold my head in a more central position by placing my head on top of my head,this gives me slight support. (263) All I do is lightly touch my neck with my right handto bring it straight. (272) 1 use my left index finger againstmy chin in order to keep my neck straight. (279) Right handto try to push chin back from twisting right and outwards. Left hand to push left temple away from left shoulder. (307) 1 would place my left index finger straight againstmy left cheek with my thumb supporting under my chin and the other fingers of my left hand curled up into a ball.

166

Dystonia -A comprehensiveand longtitudinal study in the North East of England

(317) Whilst sitting down at a table, I need to place my hand under my chin with my elbow on the table for comfort and also to disguiseit. I have been wearing a surgical collar for about six years,which I have to wrap scarvesaround for extra comfort. This enablesme to keep my headhigher. I have recentlybeento the clinic in Newcastle and they are developinga better collar for me. (340) Join both handsbehind back initially or half nelson with right hand around the back of neck. (344) When sitting watching TV, my left hand supportsmy chin which helps the head from going forward. (381) Apart from pain, I have tension in my neck that makes my head shake but it doesn'tfall to any side. I find that placing my handon my jaw steadiesthis tremor. (382) 1 use my knucklesin a half clenchedfist positionedon the left side of my face to keep my headstraight. (384) Right handusedto push right hand side of face and chin to keep head straight. (392) 1 placemy middle three fingers of my right hand on the side of my chin and push slightly, this makesmy neck feel straight, but I have over the past year discoveredthat it actually isn't straight though the pulling sensationgoes. (395) SometimesI use right hand, put straight fingers under cheekboneand thumb on index just folded back finger jaw Sometimes I to the of chin. right side of next use below hand fingertips jaw it's three my open my with on under cheekbone,sometimes in down Looking thumb to chin, with read or between writing, I supported on neck. put closedfist on right side of mouth. Watching television I lock my handsbehind my head for a while. I used to put my elbows on my kneesand cup my handsaround my chin and face to watch television or while sitting in company,but had to stop because doing it. too through shoulderswere playing up much (409) 1 alwayshad to wear a collar to keep my headup for years, nothing I did would help me. I learnedto breathein, then when I turned my headto the middle I breathed is but it does It but there watching you. work when no one out. was easier slow, (433) 1 use my right hand, gently touching or resting againstthe right hand side of my face, not all of the time but most times. (440) 1 put my left handto the left side of my face. (443) 1 place my right hand on my chin when in company, in what is supposedto appear to be a thoughtfill manner, to check on my head position. I then correct fine left jaw hand I If and my on my right accordingly. alone, occasionallyput my right hand on the upper part of the left hand side of my skull to check alignment and I adjust accordingly.

167

Dystonia -A comprehensiveand longtitudinal study in the North East of England

(460) 1 use my right hand to touch the right side of my face which helps to a certain degree. (468) Hemi-facial spasmtends to, affect the left side of the face more often than the right. But it is my right side which is injected -I am left handed - is there any by in (This has been letter) ? this answered significance note (484) 1 touch my neck on the right side with my right hand. (497) 1 circle my left arm around the back of my head and hold my left hand on the right side of my neck. (505) 1 place my left hand on my left cheek with my fingers touching my ear and my chin resting in the palm of my hand. (5 10) Propping up right side of headvAth my right hand. (538) Right hand on right side of face with elbow resting on table / desk prevented twitching andjerking of neck for first eighteenmonths. (549) By gripping the back of my neck with my right hand and using my left hand when trying to write.

168

APPENDIX G REFERENCES Bibliographical referencesshown in this thesisuse the Harvard systemof referencing. Whilst every effort has been made to ensure full compliance, some of the medical referenceshave been transferred from other referencingsystems,mainly the format of a British Medical Journal 'Original Article' in accordancewith BMJ 1996: 312: 4143., notwithstanding this, all references comply to B. S. 5605 : Bibliographical references. Altenmuller E. (1997) Causes and cures of focal limb dystonia in musicians. Performing Arts Medicine News; BAPAM. Proceedingsof the International Conference.GI. 2-GI. 12 Alter M, Kahana E, Feldman S. (1976) Differences in torsion dystonia among Israeli Ethnic groups. Advances in Neurology.- 14 ; 115-120. Barton L. (1989) Disability and dependant living. London. Falner Press. Beck A-T, Ward C.H, Mendelson M, Mock J.E. (1961) An inventory for measuring depression. Archives of General Psychiahy; 4; 561-571. Brewis M, Poskaner D. C, Rolland C, et al. (1966) Neurological disease in an English city. Acta Neurol. Scand, (Supple 24), 42: 9-89. Brin MY, Blitzer A, Stewart C. (1998) Laryngeal Dystonia (Spasmodic Dysphonia) Observations of 901 Patients and Treatment with Botulinum Toxin. In: Falm S, Marsden C.D and DeLong M. R- eds.,Advances in Neurolqy, Vol. 78: Dystonia 3. Philadelphia : LippincottRaven.237-252. Brisenden S. (1986) Independent living and the medical model of disability. Disability, Handicap andSociety. 1- 2. Abingdon. 173-178. Brooks K (1996) EuroQoI : the current state of play. Health Policy; 37; 53-72. Brown G.W, Andrews B, Harris T, Adles Z, Bridge L. (1986) Social support, self-esteem and depression. Psychol. Afed.; 16; 813-83 1. Bone M, Meltzer H. (1989) OPCS Report 3, The prevalence of disability amongst children. London. HMSO. Butler A-G. (1995) The socio-economic implications of dystonia. The Dystonia Society Neivsletter. 19 ; 4-5 and 20 ; 4-5 and 21 ; 5-6. London. TDS. Butler A. G. (1996) The social and economic implications of dystonia. European Journal of Neurology; 3: 79. Butler A-G. (1997) The epidemiology of Spasmodic Torticollis in North East England. In: The National Spasmodic Torticollis Association Annual Symposium in Nashville, Tennesseeon 9-thNovember 1997. Butler A. G, Duffey P.O.F. (1996a) The epidemiological survey of dystonia in the North East of England. European Journal ofNeurology :3: 28

169

Butler A. G, Duffey P.O.F. (1996b) An epidemiological sumey of dystonia at Darlington Memorial Hospital. European Journal ofNeurology : 3: 79 Butler A-G, Duffey P.O.F. (1997) The impact of focal dystonia on the working life of musicians. Performing Arts 11edicine News ; BAPAM. Proceedingsof the International Conference. GI. 16-GI.24. Butler A. G, Duffey P.O.F. Hawthorne M. R, Barnes M. P. (1998) The socioeconomic implications of dystonia. In: Fahn S, Marsden C.D and DeLong M. R_eds.,Advances in Neurology, Vol. 78: Dystonia 3. Philadelphia: Lippincott-Raven. 349-358. Butler A-G, Hawthorne M. R, Duffey P.O.F, Gudex C.M. (1995) A comparison using a number of different rating scales measuring the effectiveness of Botulinum Toxin therapy in the treatment of dystonia and secondary dystonic spasms. 116vementDisorders, 10 : 398. C.S.O. (1993) Life Tables. In : AnnualAbstract ofStatistics. Central Statistical Office. Claypool D. W, Duane D. D, 11strup D. M, Melton L. J. (1995) Epidemiology and Outcome of Cervical Dystonia (Spasmodic Torticollis) in Rochester, Minnesota. 1fovement Disorders, 5; 608-614. Cohen L. G, Hallett M, Geller R-D, Hochberg F. (1989) Treatment of focal dystonias of the hand with botulinum toxin injections. Journal ofNeurology, Neurosurgical Psychiatry. Dolan P, Gudex C, Kind P, et al. (1995) A social tariff for EuroQol : results from a UK general population survey. Discussion Paper 138, Centrefor Health Economics, University of York. Dolan P, Gudex C, Kind P, et al. (1996) The Time Trade-Off Method : results from a general population study. Health Economics-,5; 141-154. Duffey P.O.F, Butler A. G, Hawthorne M. R, Barnes M. P. (1998) The epidemiology of primary dystonia in the north of England. In: Fahn S. Marsden C.D. and DeLong M. R_ eds. Advancesin Neurology, Vol. 78: Dystonia 3. Philadelphia: Lippincott-Raven. 121-126. Eldridge R_ (1970) The torsion dystonias : clinical, genetic, pathological, biochemical and therapeutic aspects.Neurology; 20 ; 1-78. Elston J.S. (1997) Hemi-facial spasm. The Dystonia Society Newsletter: 27: London. TDS. 5-6. EuroQol Group (1990) EuroQol :a new facility for the measurement of health-related quality of life. Health Policy; 16; 199-208. Fahn S. (1988) Concept and classification of dystonia. In: Fahn S. Marsden C.D. Calne D.B. eds., Advancesin Neurology jO : Dystonia 2. New York. Raven Press. 1-8. Fahn S. (1995) Parkinsonism In : Rowland L. P. cd. Aferritt's textbook of neurology. 9th cd. Baltimore. Williams and Wilkins. 713-730. Fahn S, BressmanS.B, Marsden C.D. (1998) Classification of Dystonia. In: Fahn S. Marsden C.D. and DeLong M_R_ eds., Advances in Neurology, Vol 78 : Dystonia 3. Philadelphia Lippincott-Raven. 1-10. Fahn S, Marsden C.D, Calne D.B. (1987) Classification and investigation of dystonia. In: Marsden C.D, Fahn S. eds.,.Movement Disorders 2. ; London: Butterworth: 332-358. Felton BJ, Revenson.T. A, Hinrichsen G.A_ (1984) Stress and coping in the explanation of psychological adjustments among chronically sick adults. Soc. Sci. Afed; 18; 889-898.

170

Fletcher N. A_ (1990) Genetics and the dystonias. The Dystonia Society Alewsletter, TDS. 3.

London.

Fletcher N.A, Harding A-E, Marsden C.D. (1990) A genetic study of idiopathic torsion dystonia in the UK. Brain. London. Girn6nez-RolcUnS, Delgado G, Marin M, Villanueva JA, Mateo D. (1988) Hereditary torsion dystonia in Gypsies.Adv Xeurol: 50 : 73-8 1. Grandas F, Elston J, Quinn N, Marsden C.D. (1988) Blepharospasm: a review of 264 patients. Journal of Neurology Neurosurgical Psychiatry. Gudex C.M, Hawthorne M. R, Butler A-G, Duffey P.O.F. (1995) A cost-utility analysis of Botulinum Toxin therapy in the treatment of dystonia. Movement Disorders: 10 ; 373. Gudex C.M, Hawthorne M. P, Butler A. G, Duffey P.O.F. (1997) Measuring patient benefit from Botulinum Toxin in the treatment of dystonia : Feasibility of Cost-Utility Analysis. PharmacoEconomics: 12 :6; 675-684. Gudcx C.M, Hawthorne M. R, Butler A. G, Duffey P.O.F. (1998) Effect of dystonia and Botulinum Toxin treatment on health-related Quality of Life. Movement Disorders: 13 :6; 941-946. GudmundssonK. R_ (1969) The prevalence and occurrence of some rare neurological diseasesin Iceland. Acta Neurologica Scandinavia. 45.114-118. Guyatt G, Feeny D, Patrick D. (1991) Issues in quality-of-life measurement in clinical trials. Control Clinical Ttials; 12: 81S-90S. Hewer R-L. (1993) The epidemiology of disabling neurological disorders. In: Greenwood R, Barnes M. P., McMillan T.M., Ward C.D., eds., Neurol. Rehab. 4; London: Churchill Livingstone. Herz E. (1944) Dystonia L Historical review : analysis of dystonic symptoms and physiological mechanisms involved. Archives ofNeurologyantlPsychiatly. Holling'worth W, Mackenzie R, Todd CJ, ct al.(1995) Measuring changes in quality of life following magnetic resonance imaging of the knee : SF36, EuroQol or Rosser Index ? Qual. Life Res. ; 4; 325-334. Hurst N. P, Jobanputra P, Hunter M, et al. (1994) Validity of EuroQol: a generic health status instrument in patients with rheumatoid arthritis. British Journal of Rheumatoloýy; 33; 655-662. JahanshahiM. (1990) Personality in Torticollis: ; 355-363.

Changes across Time. Person. individ Diff. ; 11-4

Jahanshahi M. (1991) Psychological Factors and Depression in Torticollis. PsychosomaticResearch; 4-5 ; 493-507.

Journal of

JahanshahiM, Marsden C.D. (1988a) Personality in Torticollis :a controlled study. Psychological 1fedicine : 18; 375-387. Jahanshahi M, Marsden C.D. (1989b) Motor Disorders. In: ed-, Turpin G. The Handbook of Clinical Psychophysiology. Wiley & Sons. Chapter 19 ; 355-583. Jahanshahi M, Marsden C.D. (1990a) Body Concept, Disability and Depression in Patients with Spasmodic Torticollis. Behavioural Aleurolqy ;3; 117-131.

171

Jahanshahi M, Marsden C.D. (1990b) A Longitudinal Follow-up Study of Depression, Disability 233-246. and Body Concept in Torticollis. Behavioural Neurology; -2; Jahanshahi M, Marsden C.D. (1992) Psychological functioning before and after treatment of torticollis with botulinurn toxin. Journal of Neurology, Neurosurgery and Psychiatry ; 55 229-231. Jenkinson C, Coulter A, Wright L. (1993) Short Form 36 (SF36) Health Survey Questionnaire normative data for adults of working age. BMJ. ; 306 ; 1437-1440. Kandil MRA, Tohamy SA, Fattah HA, Ahmed HN, Farwiez HM (1994) Prevalence of chorea, dystonia and athetosis in Assuit, Egypt :a clinical and epidemiological study. Neuroepidemiology: 13 : 202-210. Korczyn A-D, Kahana E, Zilber N, Streifler M, CarassoR, Alter M. (1980) Torsion dystonia in Israel. Ann. Neurol. 8; 387-391. Kurtzke 117and Kurland L. T. (1984) The epidemiology of neurologic disease. In: Baker A. B and Baker L. H. Eds. Clinical Neurology: 4. Philadelphia: Harper and Row. 55-60. Laporte J.M. (1994) Capture-recapture techniques. RIV;

308 : 5-6.

Li S, SchoenbergB. S., Wang C, Cheng X, Rui D, Bolis C.L, Devera G, SchoenbergM. S. (1985) A prevalence study of Parkinson's Disease and other movement disorders in the Peoples Republic of China. Arch. Neurol; 42: 655-657. Ludlow C.L, Naunton R-F, Sedory S.E, Schulz G.M, Hallett M. (1989) Effects of botulinum toxin injections on speech in adductor spasmodic dysphonia. Neurology. Marsden C.D, Fahn S. (1998) Summary and Conclusions. In: Fahn S. Marsden C.D. and DeLong M. R. eds., Advances in Neurology, Vol 78 : Dystonia 3. Philadelphia : Lippincott-Raven. 359-364. Marsden C.D, Quinn N.P. (1990) The dystonias - neurological disorders affecting 20,000 people in Britain. BUJ: 300: 142. Martin J, Meltzer H, Elliott D. (1988) OPCS Report 1, The prevalence of disability amongst adults. London. I-IMSO. Martin J, White A. (1988) OPCS Report 2, The financial circumstances of disabled adults living in private households. London. HMSO. I Martin J, White A, Meltzer H. (1989) OPCS Report 4, Disabled adults employment. London. HMSO.

services, transport and

Mechanic D. (1988) Medical sociology. New York. Free Press. Meltzer H, Smyth M, RobusN. (1989) OPCS Report 6, Disabled children education. London. HMSO.

services, transport and

Measurementand Valuation of Health (MVH) Group. (1994) An interim tariff for EuroQol health states. A-feasurementand Valuation of Health (MVH) Report, York : Centre for Health Economics, University of York. Nakashinia K, Kusumi M, Inoue Y, Takahashi K. (1995) Prevalence of focal dystonias in the western area of Tottori Prefecture in Japan. XfovementDisorders; 10 : 440443.

172

NHS Management Executive. (1978) International Classification of Diseases : 9th revision. Xational Health Service (jVHS)Centrefor Coding and Classification. Loughborough. MHS Management Executive. (1993) Read Codes : Reference Manual. National Health Service (NHS) Centrefor Coding and Classification. F6002: CTP/001. Loughborough. Nutt J.G, Muenter M. D, Aronson A, Kurland L. T. (1988) Epidemiology of Dystonia in Rochester Minnesota. In: Fahn S. Marsden C.D. eds., Advances in Xeurolqy 50: Dystonia 2. New York: Raven Press:361-365. O'Brien L. G, McFetridge M. (1991) Disability : the reason for improvements in local information systems.Burisa neivsletter. 101; Hastings. 12-13. Oliver M. (1990) The politics of disablement. London. McNfillan. Oliver M. (1991) Speaking out : Disabled people and state welfare. policy. London. The Policy Studies Institute. 156-162.

In : Disability and social

Oppenheim H. (1911) Uber eine eigenartige Krampfrankheit des kindichen und jugendlichen Alters. (dysbasia lordotica progressiva, dystonia musculorurn deformans) Neurologie Centralblast.

PattersonR.M, Little S.C. (1943) Spasmodic Torticollis. Journal of Nervous and Mental Disease 98 : 571-599. Reid L(1989) Social class differences in Britain.

3rd edition. Fontana Press.

Riley D.E, Lang A. E. (1988) Dystonia. In: Kennard C. ed., Recent Advances in Clinical Aleurology. 5: 175- 200. Risch N, de Leon D, Ozelius L, et al. (1995) Genetic analysis of idiopathic torsion dystonia in Ashkenazi Jews and their recent descent from a small founder population. Mat Genet; 9: 152-159. Robinson B. C. (1983) Validation of a caregiver strain index. Journal of Gerontology, L8; 244-248. RosenbergMI

(1965) Society and the adolescent self-image. Princeton: Princeton University Press.

ScheinbergIII, Sternlieb 1. (1984) Wilson's Disease.Philadelphia. Saunders. Schoenberg B. S. (1978) Principles of neurological epidemiology. In: Schoenberg B. S. ed, Advances in Neurology L9, Neurological Epidemiology Principles and Clinical Applications. New York: Raven Press. 11-54. Schoenberg B. S. (1986) Descriptive Epidemiology of Parkinson's Disease : Disease Distribution and Hypothesis Formulation. In: Yahr M-D, Bergmann K. J. eds., Advances in Neurolqy 45, Parkinson's Disease. New York: Raven Press.277-283. Smyth M, Robus N. (1989) OPCS Report 5, The financial circumstances of disabled children living in private households. London. HMSO. Stell R, Thompson P.D,"Marsden C.D. (1988) Botulinum toxin in spasmodic torticollis. Journal of Aleurology,Neurosurgical Psychiatry. T. D. S. (1993) Diagnostic Survey of 705 members conducted between 1992 and 1993. The Dystonia Society (TDS). London.

173

U.K. Office for National Statistics, The. (1996) Estimated residential population as of 1st July 1995 Ware J.E. (1988) How to score the revised MOS Short Form health scales. Boston MA: The , Health Institute, New England Medical Center. Ware J.E, Sherbourne CD. (1992) The MOS 36-item Short Form Health Survey (SF-36) : 1. Conceptual framework and item selection jkle&cal Care, 30; 473483. . Ware J.E, Snow K. K, Kosinski M, Gandek B. (1993) SF-36 Health Survey manual and interpretation guide. Boston, MA: The Health Institute, New England Medical Center. Warr P.B, JacksonP.R- (1984) Self-Esteem and employment among young workers. Le Travail Humain; 46; 355-366. Whitaker J, Butler A. G, Barnes M. P. (1998) Botulinum Toxin treatment for people with dystonia by an outreach nurse practitioner -a comparative study. Report to the Northern and Yorkshire Regional Health Authority (NYRHA). Wood P. (1987) Maladies Imaginaires, some common misconceptions about I. C.D. H. Basle; International Disahility Stu&es. 9: 3.125-128. Wood P, Sainsbury S, Martin J, Piazza T. (1989) Researching disabilities, methodological issues. Survey1fethods Newsletter. London. 3-10. Zilber N, Korczyn A-D, Kahana E, Kalman F, Alter M. (1984) Inheritance of idiopathic torsion dystonia among Jews. Journal 11ed. Genet. 21 : 13-20.

174