Dystonia Rating Scale

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    R ating Scales for D ystonia:A M ulticenter A ssessm ent

    C ynthia L .C om ella,M D ,* Sue L eurgans,PhD ,Joanne W uu,ScM ,G lenn T .Stebbins,PhD ,T eresa C hm ura,B A and T he D ystonia S tudy G roup

    R ush-PresbyterianSt.Lukes M edical C enter,C hicago, Illinois,U SA

    A b stract:T he evaluation of dystonia requires a reliable ratingscale.T he w idely used Fahn-M arsden Scale (F-M )has notbeensufficiently tested across m ultiple centers and investigators.

    T he D ystonia Study G roup developed the U nified D ystoniaR ating Scale (U D R S) and a G lobal D ystonia R ating Scale(G D S) to serve as instrum ents to assess dystonia severity. Inthis study,25 dystonia experts evaluated the U D R S,F-M ,andG D S for internal consistency and reliability. O ne hundreddystonia patients w ere videotaped using a standardized video-tape protocol. E ach exam iner rated 20 patients using theU D R S, F-M , and G D S in random order. T he exam iner thenassessed each scale for ease of use. Statistical analysis usedC ronbachs,intraclass correlation coefficients (IC C ),gener-alized w eighted statistic, and K endalls coefficient of con-

    cordance.T he U D R S,F-M ,and G D S show ed excellentinternalconsistency (C ronbachs 0.89 0.93) and good to excellentcorrelation am ong the raters (IC C range from 0.71 0.78).Inter-

    rater agreem ent w as fair to excellent (K endalls 0.54 0.87;

    0.37 0.91) being low est for eyes,jaw , face,and larynx. T hem odifying ratings (D uration in the U D R S and Provoking Fac-tor in the F-M ) show ed less agreem entthan the m otor severityratings.A m ong scales,the totalscores correlated (Pearsons r,0.977 0.983).O verall,74% of raters found the G D S the easiestto apply. T he G D S w ith its sim plicity and ease of applicationm ay be the m ostuseful dystonia rating scale. 2002 M ove-m ent D isorder Society

    K ey w ords: dystonia;rating scale;m ovem ent disorder;out-com e assessm ent

    D ystonia is defined as a syndrom e consisting of invol-

    untary m ovem ents characterized by tw isting or sustained

    m ovem ents.1 Itis a dynam ic condition thatoften changes

    in severity depending on the posture assum ed and activ-

    ity of the involved body area. T he changing nature of

    dystonia m akes the developm ent of rating scales w ith

    acceptable clinom etric properties problem atic.2 T he

    Fahn-M arsden rating scale (F-M ) w as the firstdystonia

    scale evaluated for its clinom etric properties.In a study

    using 10 dystonia patients and 4 raters, the reliability,

    inter-rateragreem ent,and concurrent validity of the F-M

    w ere dem onstrated for the total score w ithout reporting

    the level of agreem ent for ratings of the different body

    regions.3 A lthough prom ising, the F-M scale w as never

    assessed further as a m ulti center instrum ent that could

    be used by m any investigators. Furtherm ore, the sm all

    num ber of dystonia patients included m ay not have rep-

    resented the fullspectrum of dystonia severity thatw ould

    be encountered in a m ulticenter study. S om e of the

    lim itations in the F-M include the variable definition of

    body areas,and a w eighting factor of 0.5 that halves the

    contribution of dystonia in eyes,m outh and neck to the

    total score.R ecognizing these potential lim itations,the

    D ystonia Study G roup (D SG ) designed a new rating

    scale, the U nified D ystonia R ating Scale (U D R S) that

    addressed these issues.A D S G consensus conference in

    1997 produced the U D R S and a standardized protocol

    for videotaping dystonia patient. T he global dystonia

    rating scale (G D S) w as also created.

    T he U D R S w as designed to include a m ore detailed

    assessm ent of individual body areas,including separate

    ratings for proxim aland distallim bs,and elim ination of

    the subjective patient rating for speech and sw allow ing

    included in the F-M . In addition, a duration rating w as

    developed 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: [email protected]

    M ovem ent D isordersV ol.18, N o.3,2003, pp.303312 2002 M ovem ent D isorder Society

    303

    Received 5 July 2002; Revised 29 August 2002; Accepted 29 August2002

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    A fter ratings w ere com pleted using all three scales,

    each investigator com pleted a standard questionnaire for

    each scale thatassessed the investigators opinion of ease

    of application,usefulness in an office setting and useful-

    ness in m ulticenter trials.

    Statistical A nalysis

    A nalyses w ere done using the statistical softw are SA S

    v.6.12,STA TA v.6.0,or SAS% M AG REE m acro w here

    appropriate (Stata C orp.,C ollege Station,T X ;SA S Inc.,

    C ary,N C ).T he totalscore by rater for each patientw as

    calculated for each scale. T he ratings w ere averaged

    across the five raters for each patient.S um m ary statistics

    of the overallscores are presented as m ean SD ratings

    and range, Pearsons correlation w ere used for pair-w ise

    com parison of the total scores of the three scales.

    T he internal consistency of each scale w as assessed

    by C ronbachs . O verall inter-rater agreem ent w asassessed using intraclass correlation coefficient(IC C ).

    T he IC C w as fi rst com puted for each tape (containing

    10 distinct subjects) as rated by the five raters. T he

    overallIC C w as calculated by averaging across the 10

    tapes.

    Inter-rateragreem entfor body regions w as analyzed in

    tw o w ays:K endalls coefficientof concordance and gen-

    eralized w eighted .T o show the agreem entfor com pa-

    rable body regions, the U D R S and G D S ratings for 2

    areas (proxim aland distallim bs;and jaw ,low erface and

    m outh) w ere collapsed and the m ore severe score used.

    T he K endalls coefficient of concordance provides a

    m easure of the consistency am ong raters in the rankings

    of dystonia severity. K endalls coefficient of concor-

    dance for each body region w as com puted foreach tape,

    then averaged across tapes.

    T he generalized w eighted statistic provides a

    m easure of agreem entin absolute ratings am ong m ore

    than tw o raters and on a scale w ith m ore than tw o

    rating categories.In this study, K appa w as com puted

    using four rating groupings to allow stable calcula-

    tions:G D S 0 1,23,4 6,710;U D R S 0,1,2,3 4;

    F-M 0, 1, 2, 3 4. K appa values exceeding 0.75 are

    usually considered excellent agreem ent, values be-

    tw een 0.4 and 0.75 fair to good agreem ent,and values

    below 0.4 poor agreem ent.10

    For both the K endalls coefficientof concordance and

    the generalize w eighted ,an outcom e of 0 indicates no

    agreem entbeyond chance,and 1 indicates perfectagree-

    m ent.7 R eliability and inter-rater agreem ent w ere ana-

    lyzed separately for severity and the m odifying factors

    (U D R S duration and F-M provoking factor) ratings.

    R E S U L T S

    P atients

    A total of 103 patients w ere videotaped. O ne patient

    w as excluded for failure 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 rater and U D R S data on 8

    subjects from another raterw ere m issing;these data w ere

    excluded in analyses.O ther isolated m issing item s w ere

    im puted in consultation w ith the PI.

    T here w ere 58 w om en and 42 m en w ith prim ary

    dystonia included in the study. T he patients had a m ean

    age of51 years (SD 14.8).A llform s of dystonia w ere

    represented (39 focal;37 segm entaland 24 generalized),

    and dystonic involvem ent of all body regions w as rep-

    resented. T he m ean ratings and range for each rating

    scale are show n in T able 1.

    Internal C onsistency

    Each of the three scalesw asfound to have a high levelof

    internal consistency,w ith C ronbachsranging from 0.89

    to 0.93 (Table 1).C ronbachsis a function ofthe num ber

    of item s on a rating scale and inter-rater correlation;itis an

    index ofhow stable and consistentthe item s on the scale are

    in m easuring a single characteristic such as dystonia.

    Inter-R ater A greem ent

    Each scale show ed a high level of inter-rater reliability

    for the total scores,w ith the intraclass correlation coeffi-

    cients ranging from 0.71 to 0.78 (Table 1).T he results of

    the K endalls coefficientofconcordance foreach body area

    for each scale are show n in T able 2.In general,the ratings

    form otorseverity in the U D R S and the F-M show ed higher

    levels of agreem ent than did the duration factor for the

    U D R S or the provoking factor from the F-M .T he agree-

    m ent is low est for the larynx and speech for the U D R S

    (K endalls 0.56) and for the G D S (K endalls 0.59).

    U pper face and eyes show ed the low estagreem enton the

    U D R S and the F-M .

    T A B L E 1. Sum m ary,internal consistency, and intraclasscorrelation coefficients of overall dystonia ratings for each

    rating scale

    U D RSa F-Mb G D S

    M ean SD 19.0 16.7 16.5 17.3 17.6 18.6

    R ange (2.276.4) (1.286.2) (1.685.2)C ronbachs 0.93 0.89 0.91Intraclass correlation

    coefficient 0.71 0.78 0.72

    aT en subjects had only 4 (instead of 5) ratings.bT w o subjects had only 4 (instead of 5) ratings.U D R S, U nified D ystonia R ating Scale; F-M , Fahn-M arsden Scale;

    G D S ,G lobal D ystonia R ating Scale.

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    these scales to assessdystonia.T hese results suggestthat

    the item s for each scale are consistently m easuring the

    dom ain of interest.

    T he intra-class correlation coefficients dem onstrate

    good to excellentinter-rateragreem entfor totalscoreson

    allthree scales.T his indicates that despite the individual

    differences that exist for item s w ithin a scale, ratersassign the com posite score in a sim ilar m anner.

    The inter-rater agreem ent for individualitem s (body re-

    gions) on all three scales (F-M , U D R S, G D S) and for

    m odifying ratings on tw o of the scales (F-M ,U D R S) w as

    fair to excellent using both the K endalls coefficient of

    concordance and the generalized w eighted statistic. The

    K endalls coefficient of concordance provides an estim ate

    ofthe consistency am ong raters forthe rank orderofratings

    and the assesses the exact agreem entin ratings.

    T he body regions show ing the low est level of inter-

    rater agreem ent w ere the low er face and jaw ,and upper

    face and eyes.A llthree scales show ed a sim ilar pattern,

    suggesting difficulty in the assessm ent of dystonia inthese particular anatom ical areas. It m ay be that the

    distinction betw een frequent norm al facial m ovem ents,

    such as eye blinks and facialexpression,m ay be difficult

    to distinguish from m ild interm ittent dystonia. A lterna-

    tively, itm ay be thatthe videotape exam ination outlined

    in the protocolfor these areas does notprovide sufficient

    inform ation for rating.

    Likew ise, the m odifying ratings for the U D R S (D ura-

    tion) and the F-M (Provoking Factor) show ed consistently

    low er levels of agreem entthan m otor severity ratings.T he

    m odifying factorsare com plex,com bining presence ofdys-

    tonia in particularsituations,and assessm entof m axim alor

    subm axim al intensity during the exam ination. The contri-

    bution of these m odifying ratings to the reliability of the

    rating scales is m odest,and the com plexities of these addi-

    tional ratings likely reduce the clinical usefulness of both

    the U D R S and the F-M .A lthough reliability and agreem ent

    seem equivalent am ong the three scales,the ease of appli-

    cation of the G D S reported by the m ajority of raters in this

    study suggests that this m ethod for rating of dystonia se-

    FIG . 1. Scatterplots assessing correlations of total scores betw eenU D R S, F-M ,and G D S. Pearsons r correlation coefficient.

    T A B L E 4. Investigators assessm ents of the ease ofapplication and usefulness of each dystonia rating scale in

    clinical trials and office setting

    U D R S F-M G D S

    E xtrem ely or very

    easy to apply 1/20

    5% 8/21

    38% 14/19

    74%U seful in m ulti-centerclinical trials 19/20 90% 16/21 76% 8/22 36%

    U seful in an officesetting 9/21 43% 16/21 76% 18/22 82%

    Percentage of investigators declaring specific characteristic of eachscale,am ong those w ho replied.

    R A TIN G SC A LE S F O R D YSTO N IA 307

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    verity m ay be the m ost practical to im plem ent in m ultiple

    research sites.A s w ith rating scale developm ent in other

    m ovem ent disorders,the nextsteps include an assessm ent

    of these scales for factor structure, revision of the scales

    w ith revisions to clarify rating item s and possible deletion

    of the m odifying rating of the U D R S and F-M . Tests forvalidity and responsivity to change are also necessary to

    understand the clinical utility of the scales. T he parallel

    developm ent of a teaching tape that dem onstrates the rat-

    ings foreach body area,especially face and eyes,w illincrease

    the inter-rateragreem entfor dystonia in these areas.

    A ckn ow ledgm ent:T his w ork w as supported by a grantfromthe D ystonia M edical R esearch Foundation.

    A PPE N D IX 1

    The follow ing D ystonia Study G roup sites and investigators partici-

    pated in this study:

    M .S tacy,B arrow N eurologicalInstitute,P hoenix,A Z ;D . T arsy,

    B eth Israel D eaconess M edical C enter, B oston, M A ; J.Friedm an,

    B oston M edicalC enter,B oston, M A ; L .S eeberger,C olorado N eu-

    rologicalInstitute,E nglew ood ,C O ;B .F ord,C olum bia Presbyterian

    M edical C enter, N ew Y ork, N Y ; M . E vatt, E m ory U niversity,

    A tlanta,G A ; O . Suchow ersky, Foothills H o spital,C algary,C anada;

    D . R iley, H ospital of C leveland, C leveland, O H ; M . Jog, L ondon

    H ealth Sciences C enter, L ondo n, O ntario; M .F. G ordon, L ong Is-

    land Jew ish H ospital,N ew H yde P ark, N Y ; C .A dler,M ayo C linic,

    Scottsdale, A Z ; M . B randabur,N europsych Institute, C hicago, IL ;

    M .H allett and B .K arp, N IN D S , B ethesda,M D ; S. Factor, Parkin-

    sons D isease and M ovem ent D isorders C enter of A lbany M edical

    C enter, A lbany, N Y ; D . T ruong , T he Parkinsons D isease and

    M ovem ent D isorders Institute,Fo untain V alley, C A ; R . C hen, T o-

    ronto W estern H ospital, T oronto, C anada; J.T sui, U niversity H os-

    pital, V ancouver, C anada; U . K ang, U niversity of C hicago, C hi-

    cago, IL ; A . B rashear, U niversity of Indiana, Indianapolis,IN ; M .

    Sw enson, U niversity of L ouisville, L ouisville, K Y ; P. T uite, U ni-

    versity of M innesota,M inneapolis,M N ; M . L ew and G . Petzinger,

    U niversity of S outhern C alifornia, L os A ngeles, C A ; D . T rugm an,

    U niversity of V irginia H ealth Sciences C enter,C harlottesville,V A .

    A PPE N D IX 2. D ystonia Study G roup V ideotape exam ination protocol

    A rea assessed Perspective A ctivity

    Part1: E yes and upper face C lose view of head and shoulders;sittingunsupported in chair w ithout back

    A t rest (10 sec)E yes open (10 seconds close view ,10 seconds farview )E yes closed (10 seconds close view , 10 seconds far view )Forced eye blinks:10 repetitions (10 sec)

    Part 2: L ow er face,jaw ,tongue,larynx

    P atient seated C lose view of face at rest (10 sec)R eading: standardized passage aloud (R ainbow passage).First3

    linesR epeated consonants: T ee,M ee,L a,C a:5 of each (15 sec)H olding the note eeee for 5 secondsC ount to 10 (5 sec)T ongue protrusion: (5 sec)

    O pening and closing m outh for 5 reps (10 sec)Sw allow interview Q uestion to patient:D o you have problem s w ith sw allow ing?If yes,is it occasional or frequent?D o you choke occasional or frequently?C an you sw allow firm foods? L iquids?

    Part 3:N eck Seated in chair,close view head and shoulders Frontalview atrest (instructto allow head to m ove)(10 sec)Seated w ith eyes closed (instruct to allow head to m ove) (10

    sec)Q uietconversation for 2 sentences (10 sec)T urn head all the w ay to right then leftT ilt ear to shoulder on each sideL ook up and look dow nL ateralview (5 sec)W alking back and forth tw ice (total 20 sec)

    Part 4: Shoulders and upperarm s,distal arm andhands

    Far view ofupper half ofbody A rm s extended supinated:5 secA rm s extended pronated: 5 secA rm s flexed atelbow in front of chest: 5 sec

    Finger to nose:5 repetitions (5 sec)Finger tapping,right than left: 5 reps (5 sec)Flex and extend w rists w ith arm s outstretched for 5 reps (5 sec)C up to lips,right than left arm (5 sec)W riting: Today is a nice day for 3 repetitions (m axim um tim e

    15 sec)D raw ing spiral w ithout hand resting on paper; right than left

    hand (m axim um tim e 10 sec)H old up spiral

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    A PPE N D IX 2. (C ontinued)

    A rea assessed Perspective A ctivity

    Part5: U pper leg,distalleg, foot and trunk

    Farview entire body,sitting Sitting quietly (10 sec)H eelto toe taps:5 reps on each side (10 sec)

    Far view entire body: standing and w alking Standing frontalview for 10 secStanding: lateralview for 5 secStanding: back view for 5 secW alking: aw ay and tow ard exam iner 20 feet: 2 reps (m axim um

    20 seconds)

    A PPE N D IX 3: U nified D ystonia R ating Scale (U D R S)

    Factor/area C riteria

    D uration0 N one0.5 O ccasional (25% of the tim e); predom inantly subm axim al1.0 O ccasional (25% of the tim e);predom inantly m axim al1.5 Interm ittent (2550% of the tim e); predom inantly subm axim al2.0 Interm ittent (2550% of the tim e); predom inantly m axim al2.5 F requent (5075% of the tim e); predom inantly subm axim al

    3.0 F requent (5075% of the tim e); predom inantly m axim al3.5 C onstant (75% of the tim e);predom inantly subm axim al4.0 C onstant (75% of the tim e);predom inantly m axim al

    M otor severityE yes and upper face

    0 N one1 M ild: increased blinking or slight forehead w rinkling (25% m axim alintensity)2 M oderate:eye closure w ithoutsqueezing orpronounced forehead w rinkling (25% but50%

    m axim al intensity)3 Severe:eye closure w ith squeezing,able to open eyes w ithin 10 seconds orm arked forehead

    w rinkling (50% but75% m axim al intensity)4 Extrem e:eye closure w ith squeezing,unable to open eyes w ithin 10 seconds orintense

    forehead w rinkling (75% m axim al intensity)L ow er face

    0 N one1 M ild: grim acing of low er face w ith m inim al distortion of m outh (25% m axim al)2 M oderate:grim acing oflow erface w ith m oderate distortion ofm outh (25% but50%

    m axim al)3 S evere: m arked grim acing w ith severe distortion of m outh (50% but75% m axim al)4 E xtrem e: intense grim acing w ith extrem e distortion of m outh (75% m axim al)

    Jaw and tongue0 N one1 M ild: jaw opening or tongue protrusion 25% of possible range or forced jaw clenching

    w ithout bruxism2 M oderate: jaw opening or tongue protrusion 25% but50% of possible range or forced jaw

    clenching w ith m ild bruxism secondary to dystonia3 Severe: jaw opening and/or tongue protrusion 50% but75% of possible range or forced jaw

    clenching w ith pronounced bruxism secondary to dystonia4 E xtrem e: jaw opening or tongue protrusion 75% of possible range or forced jaw clenching

    w ith inability to open m outhL arynx

    0 N one1 M ild:barely detectable hoarseness orchoked voice oroccasionalvoice breaks2 M oderate: obvious hoarseness or choked voice or frequent voice breaks

    3 S evere: m arked hoarseness or choked voice or continuous voice breaks4 E xtrem e: unable to vocalize

    N eck0 N one1 M ild: m ovem ent of head from neutral position 25% of possible norm alrange2 M oderate: m ovem ent of head from neutral position 25% but50% of possible norm alrange3 Severe: m ovem ent of head from neutral position 50% but75% of possible norm alrange4 E xtrem e: m ovem ent of head from neutral position 75% of possible norm alrange

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    A PPE N D IX 3: (C ontinued)

    Factor/area C riteria

    Shoulder and proxim al arm (right andleft)

    0 N one1 M ild: m ovem ent of shoulder or upper arm 25% of possible norm alrange2 M oderate: m ovem ent of shoulder or upper arm 25% but50% of possible norm alrange3 Severe: m ovem ent of shoulder or upper arm 50% but75% of possible norm alrange4 E xtrem e: m ovem ent of shoulder or upper arm 75% of possible norm al range

    D istal arm and hand including elbow(right and left)

    0 N one1 M ild: m ovem ent of distal arm or hand 25% of possible norm alrange2 M oderate: m ovem ent of distal arm or hand 25% but50% of possible norm alrange3 Severe: m ovem ent of distal arm or hand 50% but75% of possible norm alrange4 E xtrem e: m ovem ent of distal arm or hand 75% of possible norm al range

    Pelvis and proxim al leg (right and left)0 N one1 M ild: tilting of pelvis or m ovem ent of proxim al leg or hip 25% of possible norm alrange2 M oderate: tilting of p elvis or m ovem ent of proxim al leg or h ip 25% but50% of possible

    norm alrange3 S evere: tilting of p elvis or m ovem ent of p roxim al leg or h ip 50% but75% of possible norm al

    range4 Extrem e:tilting ofpelvis orm ovem entofproxim alleg orhip 75% ofpossible norm alrange

    D istal leg and foot including knee(right and left)

    0 N one1 M ild: m ovem ents of distal leg or foot25% of possible norm alrange2 M oderate: m ovem ents of distal leg or foot 25% but50% of possible norm alrange3 Severe: m ovem ents of distal leg or foot 50% but75% of possible norm alrange4 E xtrem e: m ovem ents of d istal leg or foot 75% of possible norm al range

    T runk0 N one1 M ild: bending of trunk 25% of possible norm alrange2 M oderate: bending of trunk 25% but50% of possible norm alrange3 Severe: bending of trunk 50% but75% of possible norm alrange4 E xtrem e: bending of trunk 75% of possible norm alrange

    A PPE N D IX 4A . F ahn M arsden rating scale

    R egion P rovoking factor S everity factor W eight P roduct

    E yes 04 04 0.5 08M outh 04 04 0.5 08S peech and sw allow 04 04 1.0 016N eck 04 04 0.5 08A rm (R ) 04 04 1.0 016A rm (L ) 04 04 1.0 016T runk 04 04 1.0 016L eg (R ) 04 04 1.0 016L eg (L ) 04 04 1.0 016Sum M ax 120

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    A PPE N D IX 4B. F ahn M arsden rating factors

    Factor/area/rating C riteria

    I. Provoking factorG eneral

    0 N o dystonia at rest or w ith action1 D ystonia only w ith particular action2 D ystonia w ith m any actions3 D ystonia on action ofdistantpartof body orinterm ittently atrest4 D ystonia present at rest

    Speech and sw allow ing1 O ccasional,either or both2 Frequent either3 Frequent one and occasional other4 Frequent both

    II. S everity factorEyes

    0 N o dystonia1 Slight: O ccasional blinking2 M ild.Frequentblinking w ithoutprolonged spasm s ofeye closure3 M oderate.Prolonged spasm s ofeyelid closure,buteyes open m ostof the tim e4 Severe.Prolonged spasm s ofeyelid closure,w ith eyes closed atleast30% of the tim e

    M outh0 N o dystonia present1 Slight.O ccasionalgrim acing or other m outh m ovem ents (e.g.,jaw opened or clenched;tongue m ovem ent2 M ild. M ovem ent present less than 50% of the tim e3 M oderate dystonic m ovem ents orcontractions presentm ostof the tim e4 Severe dystonic m ovem ents orcontractions presentm ostofthe tim e

    Speech and sw allow ing0 N orm al1 Slightly involved;speech easily understood oroccasionalchoking2 Som e difficulty in understanding speech orfrequentchoking3 M arked difficulty in understanding speech or inability to sw allow firm foods4 C om plete or alm ostcom plete anarthria,or m arked difficulty sw allow ing softfoods and liquids

    N eck0 N o dystonia present1 Slight. O ccasional pulling2 O bvious torticollis, but m ild3 M oderate pulling4 E xtrem e pulling

    A rm0 N o dystonia present1 Slightdystonia.C linically insignificant2 M ild: O bvious dystonia, but not disabling3 M oderate.A ble to grasp,w ith som e m anualfunction4 Severe.N o useful grasp

    T runk0 N o dystonia present1 Slightbending;clinically insignificant2 D efinite bending,butnotinterfering w ith standing orw alking3 M oderate bending;interfering w ith standing orw alking4 Extrem e bending oftrunk preventing standing orw alking

    L eg0 N o dystonia present1 Slightdystonia,butnotcausing im pairm ent;clinically insignificant2 M ild dystonia. W alks briskly and unaided3 M oderate dystonia.Severely im pairs w alking or requires assistance4 S evere. U nable to stand or w alk on involved leg

    R A TIN G SC A LE S F O R D YSTO N IA 311

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    A PPE N D IX 5

    T he G lobal D ystonia Severity R ating Scale

    T he global score is an overallscore for the body area.T he investi-

    gator rates the patient in relationship to all patients. If the dystoniachanges during the exam ination,the rating for the m axim aldystonia isrecorded.

    E ach body area is rated from 0 to 10:0 N o dystonia present in that body area1 M inim aldystonia5 M oderate dystonia

    10 M ostsevere dystonia

    T en body areas are tested:1) E yes and upper face,2) low er face,3) jaw and tongue,4) larynx, 5) neck,6) shoulder and proxim al arm ,7) distal arm and hand including elbow , 8) pelvis and upper leg,9) distalleg and foot,and 10) trunk.

    R E F E R E N C E S

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    2. F ahn S. A ssessm ent of the prim ary dystonias. In: M unsat TL ,editor.Q uantification of neurologicaldeficit.L ondon:B utterw orth;1989.p 241270.

    3. Burke R E, Fahn S , M arsden C D ,B ressm an SB , M oskow itz C ,Friedm an J.V alidity and reliability of a rating scale for the prim arytorsion dystonias.N eurology 1985;35:7377.

    4. C onsky ES , B asinki A , B elle L, R anaw aya R , Lang A E. T heT oronto W estern Spasm odic T orticollis R ating Scale (TW ST R S):assessm ent of the validity and inter-rater reliability. N eurology1990;40(Suppl):445.

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    6. C om ella C L , Stebbins G T, G oetz C G ,C hm ura T , B ressm an SB ,

    L ang A E. T eaching tape for the m otor section of the T oronto

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    7. A rm itage P, B erry G . S tatistical m ethods in m edical research,

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    N ew Y ork: W iley;1981.

    9. H obart JC , L am ping D L , T hom pson A J.E valuating neurological

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    chiatry 1996;60:127130.

    10. B urke R E ,Fahn S,M arsden CD .T orsion dystonia:a double-blind,

    prospective trialof high-dosage trihexyphenidyl.N eurology 1986;

    36:160 164.

    11. V olkm an J, B enecke R . D eep stim ulation for dystonia: patient

    selection and evaluation.M ov D isord 2002;17(Suppl):S112S115.

    12. V ercueilL ,K rack P,Pollak P.R esults of deep brain stim ulation for

    dystonia:a criticalreappraisal.M ov D isord 2002;17(Suppl):S89 S93.

    13. Fahn S, E lton R L , and m em bers of the U PD R S developm entcom m ittee. U nified Parkinsons disease rating scale. In: Fahn S,

    M arsden C D , C alne D B , G oldstein M , editors. R ecent develop-m ent in P arkinsons disease.V ol2.Florham Park,N J:M acm illan

    H ealth C are Inform ation; 1987.p 153164.

    14. G oetz C G , Stebbins G T, Shale H M , L ang A E, C hernik D A ,

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    scale: reliability and consistency.M ov D isord 1996;11:136 142.

    16. Stebbins G T ,G oetz C G , Flournoy T .U nified Parkinsons disease

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