Rating Scales forD ystonia:A M ulticenterA ssessm ent
Cynthia L.Com ella,M D ,*Sue Leurgans,PhD ,Joanne W uu,ScM ,G lenn T.Stebbins,PhD ,Teresa Chm ura,BA and The D ystonia Study G roup
†
Rush-Presbyterian–St.Lukes M edicalCenter,Chicago,Illinois,U SA
A bstract:
The evaluation ofdystonia requiresa reliable ratingscale.Thew idely used Fahn-M arsden Scale(F-M )hasnotbeensufficiently tested across m ultiple centers and investigators.The D ystonia Study G roup developed the U nified D ystoniaRating Scale (U D RS) and a G lobal D ystonia Rating Scale(G D S) to serve as instrum ents to assess dystonia severity.Inthisstudy,25 dystonia expertsevaluated the U D RS,F-M ,andG D S for internal consistency and reliability. O ne hundreddystonia patients w ere videotaped using a standardized video-tape protocol. Each exam iner rated 20 patients using theU D RS,F-M ,and G D S in random order.The exam iner thenassessed each scale for ease of use.Statistical analysis usedCronbach’s
,intraclass correlation coefficients (ICC),gener-alized w eighted
statistic,and K endall’s coefficientof con-cordance.TheU D RS,F-M ,and G D S show ed excellentinternalconsistency (Cronbach’s
0.89–0.93) and good to excellentcorrelation am ong theraters(ICC rangefrom 0.71–0.78).Inter-rateragreem entw as fairto excellent(K endall’s 0.54–0.87;
0.37–0.91) being low estfor eyes,jaw ,face,and larynx.Them odifying ratings (D uration in the U D RS and Provoking Fac-torin the F-M )show ed lessagreem entthan the m otorseverityratings.A m ong scales,the totalscorescorrelated (Pearson’s
r
,0.977–0.983).O verall,74% ofratersfound theG D S theeasiestto apply.The G D S w ith its sim plicity and ease ofapplicationm ay be the m ostusefuldystonia rating scale. © 2002 M ove-m entD isorderSociety
K ey w ords:
dystonia;rating scale;m ovem entdisorder;out-com e assessm ent
D ystonia isdefined asa syndrom e consisting ofinvol-untary m ovem entscharacterized by tw isting orsustainedm ovem ents.
1
Itisadynam iccondition thatoften changesin severity depending on the posture assum ed and activ-ity of the involved body area.The changing nature ofdystonia m akes the developm ent of rating scales w ithacceptable clinom etric properties problem atic.
2
TheFahn-M arsden rating scale (F-M )w as the firstdystoniascale evaluated forits clinom etric properties.In a studyusing 10 dystonia patients and 4 raters,the reliability,inter-rateragreem ent,and concurrentvalidity oftheF-M w ere dem onstrated forthe totalscore w ithoutreportingthe levelofagreem entforratings ofthe differentbodyregions.
3
A lthough prom ising,the F-M scale w as neverassessed furtheras a m ulticenterinstrum entthatcouldbe used by m any investigators.Furtherm ore,the sm allnum berofdystonia patientsincluded m ay nothave rep-resented thefullspectrum ofdystoniaseverity thatw ouldbe encountered in a m ulticenter study. Som e of thelim itations in the F-M include the variable definition ofbody areas,and a w eighting factorof0.5 thathalvesthecontribution ofdystonia in eyes,m outh and neck to thetotalscore.Recognizing these potentiallim itations,theD ystonia Study G roup (D SG ) designed a new ratingscale,the U nified D ystonia Rating Scale (U D RS) thataddressed these issues.A D SG consensus conference in1997 produced the U D RS and a standardized protocolfor videotaping dystonia patient. The global dystoniarating scale (G D S)w as also created.The U D RS w as designed to include a m ore detailedassessm entofindividualbody areas,including separateratingsforproxim aland distallim bs,and elim ination ofthe subjective patientrating forspeech and sw allow ingincluded in the F-M .In addition,a duration rating w asdeveloped that paralleled a duration factor previously
†See Appendix for a list of Study Participants.
*Corresondence to: Cynthia L. Comella, Department of Neurolog-ical Sciences, Rush Presbyterial-St.Luke's Medical Center, Suite 755,
1725 West Harrison, Chicago, IL 60612. E-mail: ccomella@rush.edu
M ovem entD isorders
V ol.18,N o.3,2003,pp.303–312© 2002 M ovem entD isorderSociety
303
Received 5 July 2002; Revised 29 August 2002; Accepted 29 August
2002
validated w ithin the Toronto W estern Spasm odic Torti-collis Scale (TW STRS).
4–6
In contrastto the F-M ,theU D RS hasno w eighting factorsforany body region.TheG D S isaglobalscaleapplied to individualbody regions.In this D SG -initiated study,the specific aim s w ere toevaluate the internal consistency, inter-rater reliabilityand clinicalapplicability of the F-M ,U D RS,and G D Sacross m ultiple sites in a large num ber of dystonia pa-tients encom passing the full spectrum of dystoniaseverity.
PA TIEN TS A N D M ETH O D SPatients
Patientsw ereincluded in thestudy ifthey had prim arydystonia and w ere follow ed in the outpatientM ovem entD isorders clinic offices atRush-Presbyterian-St.Luke’sM edicalCenter.Inform ed consent,as approved by theInstitutional Review Board at Rush Presbyterian St.Luke’s M edical Center,w as obtained from all partici-pants.The diagnosis ofprim ary dystonia w as based onpresenceofdystoniaand absenceofadditionalneurolog-icalsigns orcauses fordystonia.In particular,attentionw aspaid to recruiting patientsw ith generalized dystoniaso thateach m astertape could have adequate represen-tation oftypes and range ofdystonia.
Investigators
The 25 rating investigators from 20 institutions arelisted in A ppendix 1.The PrincipalInvestigator (CLC)w asnotincluded asa rating investigator,and carried outthe initial screening of the videotapes of all patients.There w ere no investigators from the recruiting institu-tion to preventany rater from having previous know l-edgeofthepatientsincluded forrating.Each investigatorw asaspecialistin M ovem entD isordersw ith expertiseinthe evaluation ofdystonia.
V ideotaping Protocoland D evelopm entofR ating Tapes
There w ere 103 patients videotaped using a standardvideotape protocol that incorporates and expands thevideotape protocolincluded w ith the F-M (see A ppendix2).
3
Thevideotapeprotocolincludesexam ination ofeachbody region at rest and during activation procedures.Patients w ere videotaped in a uniform m anner.A llthevideotapes w ere evaluated by the PI (CLC) w ho ratedeach of10 body areas forseverity ofdystonia using a 0to 10 scale, w ith 0 defined as no dystonia and 10 assevere dystonia.These scores w ere used to allocate pa-tients to a severity level and then random ly allocatepatientssuch thateach m asterevaluation tape included arange of dystonia severity.Each investigator rated tw om aster evaluation tapes w ith 10 patients included oneach tape,ora totalof20 patients.N o pairofinvestiga-tors rated the sam e tw o tapes.A statistician (SL) usedcom puter-generated random num bersto allocate pairsoftapes to the raters.The rating investigator view ed theevaluation videotapes a total of three tim es using theU D RS for rating during one view ing,the G D S duringanother view ing and the F-M during another view ing.The orderofscale application w as random ized.
R ating Scales
The U D RS includesratingsfor14 body areasinclud-ing eyes and upper face, low er face, jaw and tongue,larynx, neck, trunk, shoulder/proxim al arm (right andleft),distalarm /hand (rightand left),proxim alleg (rightand left),and distalleg/foot(rightand left)(A ppendix 3).Foreach ofthe 14 body areasassessed,the U D RS hasaseverity and a duration rating. The severity rating isspecific foreach body region assessed and rangesfrom 0(no dystonia)to 4 (extrem e dystonia).The duration rat-ing ism odified from theduration factoroftheTW STRS,and ranges from 0 to 4. The D uration rating assessesw hether dystonia occurs at rest or w ith action, andw hetheritis predom inantly atm axim alorsub m axim alintensity.The totalscore forthe U D RS isthe sum oftheseverity and duration factors.The m axim altotalscore ofthe U D RS is 112.The F-M rating scale (see A ppendix 4)evaluatesdys-tonia in nine body areas,including eyes,m outh,speechand sw allow ing,neck,trunk,and rightand leftarm andleg.Thearm sand legsaregiven onerating each,w ithoutdistinguishing proxim aland distalelem ents.Foreach ofthe nine body regions,severity ratingsrange from 0 (nodystonia) to 4 (severe dystonia).The provoking factorrating assesses the situation under w hich the dystoniaoccursand rangesfrom 0 (no dystonia)to 4 (dystonia atrest).The score forthe eyes,m outh,and neck are eachm ultiplied by 0.5 before being entered into the calcula-tion ofthe totalscore.The totalscore ofthe F-M is thesum of the products of the provoking, severity andw eighting factors.Them axim altotalscoreon theF-M is120.
2
The G D S ratesdystonia severity in the 14 body areasalready described forthe U D RS (see A ppendix 5).TheG D S is a Likerttype scale w ith ratings from 0 to 10 (0is no dystonia, 1 m inim al, 5 m oderate and 10 severedystonia)(A ppendix 4).There are no m odifying ratingsor w eighting factors in the G D S.The totalscore is thesum of the scores for allthe body areas.The m axim altotalscore ofthe G D S is 140.
304 C.L. CO M ELLA ET AL.
M ovem entD isorders,Vol.18,No.3,2003
A fter ratings w ere com pleted using all three scales,each investigatorcom pleted a standard questionnaire foreach scalethatassessed theinvestigator’sopinion ofeaseofapplication,usefulnessin an office setting and useful-ness in m ulticentertrials.
StatisticalA nalysis
A nalysesw ere done using the statisticalsoftw are
SASv.6.12
,
STATA v.6.0
,or
SAS% M AG REE
m acro w hereappropriate (Stata Corp.,College Station,TX ;SA S Inc.,Cary,N C).The totalscore by raterforeach patientw ascalculated for each scale. The ratings w ere averagedacrossthe five ratersforeach patient.Sum m ary statisticsofthe overallscoresare presented asm ean
SD ratingsand range,Pearson’scorrelation w ere used forpair-w isecom parison ofthe totalscores ofthe three scales.The internalconsistency ofeach scale w as assessedby Cronbach’s
. O verall inter-rater agreem ent w asassessed using intraclasscorrelation coefficient(ICC).The ICC w as firstcom puted foreach tape (containing10 distinct subjects) as rated by the five raters.TheoverallICC w ascalculated by averaging acrossthe 10tapes.Inter-rateragreem entforbody regionsw asanalyzed intw o w ays:K endall’scoefficientofconcordanceand gen-eralized w eighted
.To show the agreem entforcom pa-rable body regions,the U D RS and G D S ratings for 2areas(proxim aland distallim bs;and jaw ,low erfaceandm outh)w ere collapsed and the m ore severe score used.The K endall’s coefficientof concordance provides am easure ofthe consistency am ong ratersin the rankingsof dystonia severity. K endall’s coefficient of concor-dance foreach body region w ascom puted foreach tape,then averaged across tapes.The generalized w eighted
statistic provides am easure ofagreem entin absolute ratingsam ong m orethan tw o raters and on a scale w ith m ore than tw orating categories.In this study,K appa w as com putedusing four rating groupings to allow stable calcula-tions:G D S 0–1,2–3,4–6,7–10;U D RS 0,1,2,3–4;F-M 0, 1, 2, 3–4. K appa values exceeding 0.75 areusually considered excellent agreem ent, values be-tw een 0.4 and 0.75 fairto good agreem ent,and valuesbelow 0.4 poor agreem ent.
10
Forboth the K endall’scoefficientofconcordance andthe generalize w eighted
,an outcom e of0 indicatesnoagreem entbeyond chance,and 1 indicatesperfectagree-m ent.
7
Reliability and inter-rater agreem ent w ere ana-lyzed separately for severity and the m odifying factors(U D RS duration and F-M provoking factor)ratings.
R ESU LTSPatients
A totalof103 patients w ere videotaped.O ne patientw as excluded forfailure to com plete the videotape pro-tocol, and 2 patients w ere excluded for having otherneurological conditions besides prim ary dystonia.F-M data on 2 subjects from one raterand U D RS data on 8subjectsfrom anotherraterw erem issing;thesedataw ereexcluded in analyses.O therisolated m issing item sw ereim puted in consultation w ith the PI.There w ere 58 w om en and 42 m en w ith prim arydystonia included in the study.The patients had a m eanage of51 years(SD
14.8).A llform sofdystonia w ererepresented (39 focal;37 segm entaland 24 generalized),and dystonic involvem entof allbody regions w as rep-resented. The m ean ratings and range for each ratingscale are show n in Table 1.
InternalC onsistency
Each ofthethreescalesw asfound to haveahigh levelofinternalconsistency,w ith Cronbach’s
ranging from 0.89to 0.93 (Table1).Cronbach’s
isafunction ofthenum berofitem son arating scaleand inter-ratercorrelation;itisanindexofhow stableandconsistenttheitem sonthescalearein m easuring a single characteristic such asdystonia.
Inter-R ater A greem ent
Each scale show ed a high levelofinter-raterreliabilityfor the totalscores,w ith the intraclass correlation coeffi-cients ranging from 0.71 to 0.78 (Table 1).The results oftheK endall’scoefficientofconcordanceforeach body areaforeach scale are show n in Table 2.In general,the ratingsform otorseverity in theU D RS and theF-M show ed higherlevels of agreem ent than did the duration factor for theU D RS orthe provoking factorfrom the F-M .The agree-m entis low estfor the larynx and speech for the U D RS(K endall’s
0.56)and forthe G D S (K endall’s
0.59).U pperface and eyes show ed the low estagreem enton theU D RS and the F-M .
TA BLE 1.
Sum m ary,internalconsistency,and intraclasscorrelation coefficients ofoveralldystonia ratings for eachrating scale
U D RS
a
F-M
b
G D SM ean
SD 19.0
16.7 16.5
17.3 17.6
18.6Range (2.2–76.4) (1.2–86.2) (1.6–85.2)Cronbach’s
0.93 0.89 0.91Intraclass correlationcoefficient 0.71 0.78 0.72
a
Ten subjects had only 4 (instead of5)ratings.
b
Tw o subjects had only 4 (instead of5)ratings.U D RS,U nified D ystonia Rating Scale;F-M ,Fahn-M arsden Scale;G D S,G lobalD ystonia Rating Scale.
RATING SCALES FO R D YSTO NIA 305
M ovem entD isorders,Vol.18,No.3,2003
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