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M ovem ent D isorders

V ol.18,N o.3,2003,pp.303–312
© 2002 M ovem ent D isorder Society

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 Study G roup†

R ush-P resbyterian–St.Lukes M edical C enter,C hicago,Illinois,U SA

A bstract: T he evaluation of dystonia requires a reliable rating cordance.T he U D R S,F-M ,and G D S show ed excellentinternal
scale.T he w idely used Fahn-M arsden Scale (F-M )has notbeen consistency (C ronbach’s ! 0.89 – 0.93) and good to excellent
sufficiently tested across m ultiple centers and investigators. correlation am ong the raters (IC C range from 0.71– 0.78).Inter-
T he D ystonia Study G roup developed the U nified D ystonia rater agreem ent w as fair to excellent (K endall’s 0.54 – 0.87; "
R ating Scale (U D R S) and a G lobal D ystonia R ating Scale 0.37– 0.91) being low est for eyes, jaw , face, and larynx. T he
(G D S) to serve as instrum ents to assess dystonia severity. In m odifying ratings (D uration in the U D R S and Provoking Fac-
this study,25 dystonia experts evaluated the U D R S,F-M ,and tor in the F-M ) show ed less agreem entthan the m otor severity
G D S for internal consistency and reliability. O ne hundred ratings.A m ong scales,the totalscores correlated (Pearson’s r,
dystonia patients w ere videotaped using a standardized video- 0.977– 0.983).O verall,74% ofraters found the G D S the easiest
tape protocol. E ach exam iner rated 20 patients using the to apply.T he G D S w ith its sim plicity and ease of application
U D R S, F-M , and G D S in random order. T he exam iner then m ay be the m ost useful dystonia rating scale. © 2002 M ove-
assessed each scale for ease of use. Statistical analysis used m ent D isorder Society
C ronbach’s !,intraclass correlation coefficients (IC C ),gener- K ey w ords: dystonia;rating scale;m ovem entdisorder;out-
alized w eighted " statistic, and K endall’s coefficient of con- com e assessm ent

D ystonia is defined as a syndrom e consisting ofinvol- assessed further as a m ulti center instrum ent that could
untary m ovem ents characterized by tw isting orsustained be used by m any investigators. Furtherm ore, the sm all
m ovem ents.1 Itis a dynam ic condition thatoften changes num ber of dystonia patients included m ay nothave rep-
in severity depending on the posture assum ed and activ- resented the fullspectrum ofdystonia severity thatw ould
ity of the involved body area. T he changing nature of be encountered in a m ulticenter study. Som e of the
dystonia m akes the developm ent of rating scales w ith lim itations in the F-M include the variable definition of
acceptable clinom etric properties problem atic.2 T he body areas,and a w eighting factor of 0.5 thathalves the
Fahn-M arsden rating scale (F-M ) w as the first dystonia contribution of dystonia in eyes,m outh and neck to the
scale evaluated for its clinom etric properties.In a study total score. R ecognizing these potential lim itations, the
using 10 dystonia patients and 4 raters, the reliability, D ystonia Study G roup (D SG ) designed a new rating
inter-rateragreem ent,and concurrentvalidity ofthe F-M scale, the U nified D ystonia R ating Scale (U D R S) that
w ere dem onstrated for the total score w ithout reporting addressed these issues.A D SG consensus conference in
the level of agreem ent for ratings of the different body 1997 produced the U D R S and a standardized protocol
regions.3 A lthough prom ising,the F-M scale w as never 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
† See Appendix for a list of Study Participants.
*Corresondence to: Cynthia L. Comella, Department of Neurolog- assessm ent of individual body areas, including separate
ical Sciences, Rush Presbyterial-St.Luke's Medical Center, Suite 755, ratings for proxim aland distallim bs,and elim ination of
1725 West Harrison, Chicago, IL 60612. E-mail: ccomella@rush.edu the subjective patient rating for speech and sw allow ing
Received 5 July 2002; Revised 29 August 2002; Accepted 29 August
2002 included in the F-M . In addition, a duration rating w as
developed that paralleled a duration factor previously

303
304 C .L. C O M E LLA E T A L.

validated w ithin the T oronto W estern Spasm odic T orti- range of dystonia severity. E ach investigator rated tw o
collis Scale (T W ST R S).4 – 6 In contrast to the F-M , the m aster evaluation tapes w ith 10 patients included on
U D R S has no w eighting factors forany body region.T he each tape,or a totalof 20 patients.N o pair of investiga-
G D S is a globalscale applied to individualbody regions. tors rated the sam e tw o tapes. A statistician (SL ) used
In this D SG -initiated study,the specific aim s w ere to com puter-generated random num bers to allocate pairs of
evaluate the internal consistency, inter-rater reliability tapes to the raters. T he rating investigator view ed the
and clinical applicability of the F-M , U D R S, and G D S evaluation videotapes a total of three tim es using the
across m ultiple sites in a large num ber of dystonia pa- U D R S for rating during one view ing, the G D S during
tients encom passing the full spectrum of dystonia another view ing and the F-M during another view ing.
severity. T he order of scale application w as random ized.

P A T IE N T S A N D M E T H O D S
R ating Scales
P atients T he U D R S includes ratings for 14 body areas includ-
Patients w ere included in the study ifthey had prim ary ing eyes and upper face, low er face, jaw and tongue,
dystonia and w ere follow ed in the outpatientM ovem ent larynx, neck, trunk, shoulder/proxim al arm (right and
D isorders clinic offices at R ush-Presbyterian-St.L uke’s left),distalarm /hand (rightand left),proxim alleg (right
M edical C enter. Inform ed consent, as approved by the and left),and distalleg/foot(rightand left)(A ppendix 3).
Institutional R eview B oard at R ush Presbyterian St. For each of the 14 body areas assessed,the U D R S has a
L uke’s M edical C enter, w as obtained from all partici- severity and a duration rating. T he severity rating is
pants. T he diagnosis of prim ary dystonia w as based on specific foreach body region assessed and ranges from 0
presence ofdystonia and absence ofadditionalneurolog- (no dystonia) to 4 (extrem e dystonia).T he duration rat-
ical signs or causes for dystonia.In particular,attention ing is m odified from the duration factorofthe T W ST R S,
w as paid to recruiting patients w ith generalized dystonia and ranges from 0 to 4. T he D uration rating assesses
so that each m aster tape could have adequate represen- w hether dystonia occurs at rest or w ith action, and
tation of types and range of dystonia. w hether it is predom inantly at m axim al or sub m axim al
intensity.T he totalscore for the U D R S is the sum of the
Investigators severity and duration factors.T he m axim altotalscore of
T he 25 rating investigators from 20 institutions are the U D R S is 112.
listed in A ppendix 1. T he Principal Investigator (C L C ) T he F-M rating scale (see A ppendix 4) evaluates dys-
w as notincluded as a rating investigator,and carried out tonia in nine body areas,including eyes,m outh,speech
the initial screening of the videotapes of all patients. and sw allow ing,neck,trunk,and rightand leftarm and
T here w ere no investigators from the recruiting institu- leg.T he arm s and legs are given one rating each,w ithout
tion to prevent any rater from having previous know l- distinguishing proxim aland distalelem ents.For each of
edge ofthe patients included forrating.E ach investigator the nine body regions,severity ratings range from 0 (no
w as a specialistin M ovem entD isorders w ith expertise in dystonia) to 4 (severe dystonia). T he provoking factor
the evaluation of dystonia. rating assesses the situation under w hich the dystonia
occurs and ranges from 0 (no dystonia) to 4 (dystonia at
V ideotaping P rotocol and D evelopm ent rest).T he score for the eyes,m outh,and neck are each
of R ating T apes m ultiplied by 0.5 before being entered into the calcula-
T here w ere 103 patients videotaped using a standard tion of the total score.T he total score of the F-M is the
videotape protocol that incorporates and expands the sum of the products of the provoking, severity and
videotape protocolincluded w ith the F-M (see A ppendix w eighting factors.T he m axim altotalscore on the F-M is
2).3 T he videotape protocolincludes exam ination ofeach 120.2
body region at rest and during activation procedures. T he G D S rates dystonia severity in the 14 body areas
Patients w ere videotaped in a uniform m anner. A ll the already described for the U D R S (see A ppendix 5).T he
videotapes w ere evaluated by the PI (C L C ) w ho rated G D S is a L ikert type scale w ith ratings from 0 to 10 (0
each of 10 body areas for severity of dystonia using a 0 is no dystonia, 1 m inim al, 5 m oderate and 10 severe
to 10 scale, w ith 0 defined as no dystonia and 10 as dystonia) (A ppendix 4).T here are no m odifying ratings
severe dystonia.T hese scores w ere used to allocate pa- or w eighting factors in the G D S. T he total score is the
tients to a severity level and then random ly allocate sum of the scores for all the body areas. T he m axim al
patients such thateach m asterevaluation tape included a total score of the G D S is 140.

M ovem ent D isorders,V ol.18,N o.3,2003


R A TIN G SC A LE S F O R D YSTO N IA 305

A fter ratings w ere com pleted using all three scales, T A B L E 1. Sum m ary,internal consistency,and intraclass
each investigator com pleted a standard questionnaire for correlation coefficients of overall dystonia ratings for each
rating scale
each scale thatassessed the investigator’s opinion ofease
of application,usefulness in an office setting and useful- U D R Sa F-M b
GDS
ness in m ulticenter trials. M ean # SD 19.0 # 16.7 16.5 # 17.3 17.6 # 18.6
R ange (2.2–76.4) (1.2–86.2) (1.6–85.2)
C ronbach’s ! 0.93 0.89 0.91
Statistical A nalysis Intraclass correlation
coefficient 0.71 0.78 0.72
A nalyses w ere done using the statisticalsoftw are SA S
v.6.12,STA TA v.6.0,or SA S% M A G R E E m acro w here a
T en subjects had only 4 (instead of 5) ratings.
b
appropriate (Stata C orp.,C ollege Station,T X ;SA S Inc., T 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;
C ary,N C ).T he totalscore by rater for each patientw as G D S,G lobal D ystonia R ating Scale.
calculated for each scale. T he ratings w ere averaged
across the five raters foreach patient.Sum m ary statistics R E SU L T S
ofthe overallscores are presented as m ean # SD ratings
P atients
and range,Pearson’s correlation w ere used for pair-w ise
com parison of the total scores of the three scales. A total of 103 patients w ere videotaped. O ne patient
T he internalconsistency of each scale w as assessed w as excluded for failure to com plete the videotape pro-
by C ronbach’s !. O verall inter-rater agreem ent w as tocol, and 2 patients w ere excluded for having other
assessed using intraclass correlation coefficient(IC C ). neurological conditions besides prim ary dystonia. F-M
T he IC C w as first com puted for each tape (containing data on 2 subjects from one rater and U D R S data on 8
10 distinct subjects) as rated by the five raters. T he subjects from anotherraterw ere m issing;these data w ere
overallIC C w as calculated by averaging across the 10 excluded in analyses.O ther isolated m issing item s w ere
im puted in consultation w ith the PI.
tapes.
T here w ere 58 w om en and 42 m en w ith prim ary
Inter-rateragreem entforbody regions w as analyzed in
dystonia included in the study.T he patients had a m ean
tw o w ays:K endall’s coefficientofconcordance and gen-
age of51 years (SD $ 14.8).A llform s ofdystonia w ere
eralized w eighted ".T o show the agreem entfor com pa-
represented (39 focal;37 segm entaland 24 generalized),
rable body regions, the U D R S and G D S ratings for 2
and dystonic involvem ent of all body regions w as rep-
areas (proxim aland distallim bs;and jaw ,low erface and resented. T he m ean ratings and range for each rating
m outh) w ere collapsed and the m ore severe score used. scale are show n in T able 1.
T he K endall’s coefficient of concordance provides a
m easure of the consistency am ong raters in the rankings Internal C onsistency
of dystonia severity. K endall’s coefficient of concor- Each ofthe three scalesw asfound to have a high levelof
dance for each body region w as com puted for each tape, internalconsistency,w ith C ronbach’s !ranging from 0.89
then averaged across tapes. to 0.93 (Table 1).C ronbach’s !is a function ofthe num ber
T he generalized w eighted " statistic provides a ofitem s on a rating scale and inter-ratercorrelation;itis an
m easure of agreem entin absolute ratings am ong m ore index ofhow stable and consistentthe item son the scale are
than tw o raters and on a scale w ith m ore than tw o in m easuring a single characteristic such as dystonia.
rating categories. In this study, K appa w as com puted Inter-R ater A greem ent
using four rating groupings to allow stable calcula- Each scale show ed a high level of inter-rater reliability
tions: G D S 0 –1,2–3,4 – 6,7–10; U D R S 0,1,2,3– 4; for the total scores, w ith the intraclass correlation coeffi-
F-M 0, 1, 2, 3– 4. K appa values exceeding 0.75 are cients ranging from 0.71 to 0.78 (Table 1).The results of
usually considered excellent agreem ent, values be- the K endall’s coefficientofconcordance foreach body area
tw een 0.4 and 0.75 fair to good agreem ent,and values foreach scale are show n in Table 2.In general,the ratings
below 0.4 poor agreem ent.10 form otorseverity in the U D R S and the F-M show ed higher
For both the K endall’s coefficientof concordance and levels of agreem ent than did the duration factor for the
the generalize w eighted ",an outcom e of 0 indicates no U D R S or the provoking factor from the F-M .The agree-
agreem entbeyond chance,and 1 indicates perfectagree- m ent is low est for the larynx and speech for the U D R S
m ent.7 R eliability and inter-rater agreem ent w ere ana- (K endall’s $ 0.56) and for the G D S (K endall’s $ 0.59).
lyzed separately for severity and the m odifying factors U pper face and eyes show ed the low estagreem enton the
(U D R S duration and F-M provoking factor) ratings. U D R S and the F-M .

M ovem ent D isorders,V ol.18,N o.3,2003


306 C .L. C O M E LLA E T A L.

T A B L E 2. A greem ent of raters for m otor severity ratings of different body


regions: K endall’s coefficient of concordance
U D RS F-M
Provoking
B ody region Severity D uration Severity factor GDS
L eg 0.76 0.73 0.75 0.72 0.75
T runk 0.74 0.71 0.78 0.72 0.75
A rm 0.70 0.54 0.78 0.73 0.74
N eck 0.85 0.67 0.87 0.64 0.86
L arynx/speech 0.56 0.52 0.71 0.71 0.59
L ow er face and jaw 0.68 0.64 0.71 0.65 0.73
U pper face and eyes 0.63 0.57 0.59 0.55 0.65

The generalized w eighted "statistic foreach rating scale valid rating scales. T he testing of new therapeutic ap-
in each body area isshow n in Table 3.In general,the m otor proaches to dystonia, including surgical interventions,
severity ratings ofboth the U D R S and F-M show ed greater w ill require collaboration am ong m ultiple investigators
agreem entthan the duration or provoking factors.A s seen and study sites. T o im plem ent m ulticenter studies, a
previously w ith the K endall’s,agreem entam ong raters w as reliable and valid instrum entthatcan assess the spectrum
low est for upper face and eyes (m otor severity of U D R S of dystonia severity is necessary.9 T he F-M dystonia
"$ 0.52;F-M "$ 0.52;G D S "$ 0.58). rating scale has been used as the standard outcom e m ea-
sure.10 –12 O nly one sm all study, how ever, has dem on-
P airw ise C om parisons and P earson’s C orrelations
strated reliability and validity of the F-M .3 W hether this
T he total scores for the three scales are highly corre- rating scale is usefulforlarge clinicaltrials had notbeen
lated w ith each other.T he three pairw ise scatterplots are assessed until the current study.
show n in Figure 1.T he scales had Pearson’s correlations
T he U D R S w as developed to address the perceived
of0.983 (U D R S and G D S),0.977 (F-M and U D R S),and
lim itations of the F-M . T he U D R S divides the body
0.980 (F-M and U D R S).
regions into sm aller m ore defined areas, adding a new
Investigator Q uestionnaires m odifying rating scale that w as successfully used for
T he results ofthe investigatorquestionnaire are show n focaldystonia (T W ST R S),4 – 6 and elim inates the w eight-
in T able 4.Seventy-four percentof the rating investiga- ing factor that lessened the contribution of dystonia se-
tors found the G D S extrem ely or very easy to apply and verity in certain body regions in the F-M scale.T he G D S
82% found itusefulform easuring dystonia severity in an w as designed as a sim ple direct assessm ent of overall
office setting.In contrast,only 5% ofinvestigators found severity of dystonia in each body area. D espite differ-
the U D R S easy to use,and 38% the F-M . ences in scale construction, the m easures of internal
consistency and inter-raterreliability w ere w ithin accept-
D ISC U SSIO N able range forallthree scales.T he C ronbach’s !m ay be
T he m easurem entof dystonia severity lies w ith clini- som ew hatinflated because ofthe num berofitem s w ithin
cal exam ination and the developm ent of reliable and each scale,butthe results suggesta stable construction of

T A B L E 3. A greem ent of raters for m otor severity ratings of different body regions:
G eneralized w eighted !
U D RS F-M
Provoking
B ody region Severity D uration Severity factor GDS
L eg 0.87 0.80 0.91 0.85 0.80
T runk 0.90 0.75 0.88 0.81 0.86
A rm 0.82 0.44 0.90 0.89 0.83
N eck 0.81 0.74 0.84 0.51 0.82
L arynx/speech 0.66 0.44 0.82 0.77 0.82
L ow er face and jaw 0.63 0.49 0.62 0.61 0.73
U pper face and eyes 0.52 0.43 0.52 0.37 0.58

M ovem ent D isorders,V ol.18,N o.3,2003


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

T A B L E 4. Investigators’ assessm ents of the ease of


application and usefulness of each dystonia rating scale in
clinical trials and office setting
U D RS F-M GDS
E xtrem ely or very
easy to apply 1/20 $ 5% 8/21 $ 38% 14/19 $ 74%
U seful in m ulti-center
clinical trials 19/20 $ 90% 16/21 $ 76% 8/22 $ 36%
U seful in an office
setting 9/21 $ 43% 16/21 $ 76% 18/22 $ 82%

Percentage of investigators declaring specific characteristic of each


scale,am ong those w ho replied.

these scales to assess dystonia.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 entfortotalscores on
allthree scales.T his indicates thatdespite the individual
differences that exist for item s w ithin a scale, raters
assign the com posite score in a sim ilar m anner.
The inter-rater agreem entfor 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 endall’s coefficient of
concordance and the generalized w eighted "statistic.The
K endall’s coefficientof concordance provides an estim ate
ofthe consistency am ong ratersforthe rank orderofratings
and the "assesses the exactagreem entin ratings.
T he body regions show ing the low est level of inter-
rater agreem entw 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 in
these 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 protocolforthese 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.The
m odifying factors are com plex,com bining presence ofdys-
tonia in particularsituations,and assessm entofm 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 ofthese 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 equivalentam ong the three scales,the ease of appli-
F IG . 1. Scatterplots assessing correlations of total scores betw een
U D R S,F-M ,and G D S.Pearson’s r correlation coefficient. cation ofthe G D S reported by the m ajority ofraters in this
study suggests that this m ethod for rating of dystonia se-

M ovem ent D isorders,V ol.18,N o.3,2003


308 C .L. C O M E LLA E T A L.

verity m ay be the m ostpracticalto im plem entin m ultiple M .Stacy,B arrow N eurologicalInstitute,Phoenix,A Z ;D .T arsy,
research sites. A s w ith rating scale developm ent in other B eth Israel D eaconess M edical C enter, B oston, M A ; J. Friedm an,
B oston M edical C enter,B oston,M A ; L .Seeberger,C olorado N eu-
m ovem entdisorders,the nextsteps include an assessm ent rologicalInstitute,E nglew ood,C O ;B .Ford,C olum bia Presbyterian
of these scales for factor structure, revision of the scales M edical C enter, N ew Y ork, N Y ; M . E vatt, E m ory U niversity,
w ith revisions to clarify rating item s and possible deletion A tlanta,G A ;O .Suchow ersky,Foothills H ospital,C algary,C anada;
of the m odifying rating of the U D R S and F-M .Tests for D . R iley, H ospital of C leveland, C leveland, O H ; M . Jog, L ondon
H ealth Sciences C enter, L ondon, O ntario; M .F. G ordon, L ong Is-
validity and responsivity to change are also necessary to land Jew ish H ospital,N ew H yde Park,N Y ; C .A dler,M ayo C linic,
understand the clinical utility of the scales. The parallel Scottsdale, A Z ; M . B randabur, N europsych Institute, C hicago, IL ;
developm ent of a teaching tape that dem onstrates the rat- M . H allett and B . K arp, N IN D S, B ethesda, M D ; S. Factor, Parkin-
ingsforeach body area,especially face and eyes,w illincrease son’s 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 Parkinson’s D isease and
the inter-rateragreem entfordystonia in these areas. M ovem ent D isorders Institute, Fountain 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-
A cknow ledgm ent:T his w ork w as supported by a grantfrom
pital, V ancouver, C anada; U . K ang, U niversity of C hicago, C hi-
the D ystonia M edical R esearch Foundation.
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-
A P P E N D IX 1
versity of M innesota,M inneapolis,M N ; M .L ew and G .Petzinger,
The follow ing D ystonia Study G roup sites and investigators partici- U niversity of Southern C alifornia, L os A ngeles, C A ; D . T rugm an,
pated in this study: U niversity of V irginia H ealth Sciences C enter,C harlottesville,V A .

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


A rea assessed Perspective A ctivity
Part 1: E yes and upper face C lose view of head and shoulders; sitting A t rest (10 sec)
unsupported in chair w ithout back E yes open (10 seconds close view ,10 seconds far view )
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 , Patient seated C lose view of face at rest (10 sec)
tongue,larynx R eading: standardized passage aloud (R ainbow passage).First 3
lines
R epeated consonants: T ee,M ee,L a,C a: 5 of each (15 sec)
H olding the note “eeee” for 5 seconds
C 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 Frontal view at rest (instruct to allow head to m ove) (10 sec)
Seated w ith eyes closed (instruct to allow head to m ove) (10
sec)
Q uiet conversation for 2 sentences (10 sec)
T urn head all the w ay to right then left
T ilt ear to shoulder on each side
L ook up and look dow n
L ateral view (5 sec)
W alking back and forth tw ice (total 20 sec)
Part 4: Shoulders and upper Far view of upper half of body A rm s extended supinated: 5 sec
arm s,distal arm and A rm s extended pronated: 5 sec
hands A rm s flexed at elbow 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: “T oday 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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R A TIN G SC A LE S F O R D YSTO N IA 309

A P P E N D IX 2. (C ontinued)
A rea assessed Perspective A ctivity
Part 5: U pper leg,distal Far view entire body,sitting Sitting quietly (10 sec)
leg,foot and trunk H eel to toe taps: 5 reps on each side (10 sec)
Far view entire body: standing and w alking Standing frontal view for 10 sec
Standing: lateral view for 5 sec
Standing: back view for 5 sec
W alking: aw ay and tow ard exam iner 20 feet: 2 reps (m axim um
20 seconds)

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


Factor/area C riteria
D uration
0 N one
0.5 O ccasional (%25% of the tim e); predom inantly subm axim al
1.0 O ccasional (%25% of the tim e); predom inantly m axim al
1.5 Interm ittent (25–50% of the tim e); predom inantly subm axim al
2.0 Interm ittent (25–50% of the tim e); predom inantly m axim al
2.5 Frequent (50–75% of the tim e); predom inantly subm axim al
3.0 Frequent (50–75% of the tim e); predom inantly m axim al
3.5 C onstant (&75% of the tim e); predom inantly subm axim al
4.0 C onstant (&75% of the tim e); predom inantly m axim al
M otor severity
E yes and upper face
0 N one
1 M ild: increased blinking or slight forehead w rinkling (" 25% m axim al intensity)
2 M oderate: eye closure w ithout squeezing or pronounced forehead w rinkling (&25% but " 50%
m axim al intensity)
3 Severe: eye closure w ith squeezing,able to open eyes w ithin 10 seconds or m arked forehead
w rinkling (&50% but " 75% m axim al intensity)
4 E xtrem e: eye closure w ith squeezing,unable to open eyes w ithin 10 seconds or intense
forehead w rinkling (&75% m axim al intensity)
L ow er face
0 N one
1 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 of low er face w ith m oderate distortion of m outh (&25% but " 50%
m axim al)
3 Severe: m arked grim acing w ith severe distortion of m outh (&50% but " 75% m axim al)
4 E xtrem e: intense grim acing w ith extrem e distortion of m outh (&75% m axim al)
Jaw and tongue
0 N one
1 M ild: jaw opening or tongue protrusion " 25% of possible range or forced jaw clenching
w ithout bruxism
2 M oderate: jaw opening or tongue protrusion &25% but " 50% of possible range or forced jaw
clenching w ith m ild bruxism secondary to dystonia
3 Severe: jaw opening and/or tongue protrusion &50% but " 75% of possible range or forced jaw
clenching w ith pronounced bruxism secondary to dystonia
4 E xtrem e: jaw opening or tongue protrusion &75% of possible range or forced jaw clenching
w ith inability to open m outh
L arynx
0 N one
1 M ild: barely detectable hoarseness or choked voice or occasional voice breaks
2 M oderate: obvious hoarseness or choked voice or frequent voice breaks
3 Severe: m arked hoarseness or choked voice or continuous voice breaks
4 E xtrem e: unable to vocalize
N eck
0 N one
1 M ild: m ovem ent of head from neutral position " 25% of possible norm al range
2 M oderate: m ovem ent of head from neutral position &25% but " 50% of possible norm al range
3 Severe: m ovem ent of head from neutral position &50% but " 75% of possible norm al range
4 E xtrem e: m ovem ent of head from neutral position &75% of possible norm al range

M ovem ent D isorders,V ol.18,N o.3,2003


310 C .L. C O M E LLA E T A L.

A P P E N D IX 3: (C ontinued)
Factor/area C riteria
Shoulder and proxim al arm (right and
left)
0 N one
1 M ild: m ovem ent of shoulder or upper arm " 25% of possible norm al range
2 M oderate: m ovem ent of shoulder or upper arm 25% but" 50% of possible norm al range
3 Severe: m ovem ent of shoulder or upper arm 50% but" 75% of possible norm al range
4 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 one
1 M ild: m ovem ent of distal arm or hand " 25% of possible norm al range
2 M oderate: m ovem ent of distal arm or hand 25% but" 50% of possible norm al range
3 Severe: m ovem ent of distal arm or hand 50% but" 75% of possible norm al range
4 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 one
1 M ild: tilting of pelvis or m ovem ent of proxim al leg or hip " 25% of possible norm al range
2 M oderate: tilting of pelvis or m ovem ent of proxim al leg or hip 25% but" 50% of possible
norm al range
3 Severe: tilting of pelvis or m ovem ent of proxim al leg or hip 50% but" 75% of possible norm al
range
4 E xtrem e: tilting of pelvis or m ovem ent of proxim al leg or hip 75% of possible norm al range
D istal leg and foot including knee
(right and left)
0 N one
1 M ild: m ovem ents of distal leg or foot" 25% of possible norm al range
2 M oderate: m ovem ents of distal leg or foot 25% but" 50% of possible norm al range
3 Severe: m ovem ents of distal leg or foot 50% but" 75% of possible norm al range
4 E xtrem e: m ovem ents of distal leg or foot 75% of possible norm al range
T runk
0 N one
1 M ild: bending of trunk " 25% of possible norm al range
2 M oderate: bending of trunk 25% but " 50% of possible norm al range
3 Severe: bending of trunk &50% but " 75% of possible norm al range
4 E xtrem e: bending of trunk &75% of possible norm al range

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


R egion Provoking factor Severity factor W eight Product
E yes 0–4 '0–4 0.5 0–8
M outh 0–4 '0–4 0.5 0–8
Speech and sw allow 0–4 '0–4 1.0 0–16
N eck 0–4 '0–4 0.5 0–8
A rm (R ) 0–4 '0–4 1.0 0–16
A rm (L ) 0–4 '0–4 1.0 0–16
T runk 0–4 '0–4 1.0 0–16
L eg (R ) 0–4 '0–4 1.0 0–16
L eg (L ) 0–4 '0–4 1.0 0–16
Sum M ax 120

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

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


Factor/area/rating C riteria
I. Provoking factor
G eneral
0 N o dystonia at rest or w ith action
1 D ystonia only w ith particular action
2 D ystonia w ith m any actions
3 D ystonia on action of distant part of body or interm ittently at rest
4 D ystonia present at rest
Speech and sw allow ing
1 O ccasional,either or both
2 Frequent either
3 Frequent one and occasional other
4 Frequent both
II. Severity factor
E yes
0 N o dystonia
1 Slight: O ccasional blinking
2 M ild.Frequent blinking w ithout prolonged spasm s of eye closure
3 M oderate.Prolonged spasm s of eyelid closure,but eyes open m ost of the tim e
4 Severe.Prolonged spasm s of eyelid closure,w ith eyes closed at least 30% of the tim e
M outh
0 N o dystonia present
1 Slight.O ccasional grim acing or other m outh m ovem ents (e.g.,jaw opened or clenched; tongue m ovem ent
2 M ild.M ovem ent present less than 50% of the tim e
3 M oderate dystonic m ovem ents or contractions present m ost of the tim e
4 Severe dystonic m ovem ents or contractions present m ost of the tim e
Speech and sw allow ing
0 N orm al
1 Slightly involved; speech easily understood or occasional choking
2 Som e difficulty in understanding speech or frequent choking
3 M arked difficulty in understanding speech or inability to sw allow firm foods
4 C om plete or alm ost com plete anarthria,or m arked difficulty sw allow ing soft foods and liquids
N eck
0 N o dystonia present
1 Slight.O ccasional pulling
2 O bvious torticollis,but m ild
3 M oderate pulling
4 E xtrem e pulling
A rm
0 N o dystonia present
1 Slight dystonia.C linically insignificant
2 M ild: O bvious dystonia,but not disabling
3 M oderate.A ble to grasp,w ith som e m anual function
4 Severe.N o useful grasp
T runk
0 N o dystonia present
1 Slight bending; clinically insignificant
2 D efinite bending,but not interfering w ith standing or w alking
3 M oderate bending; interfering w ith standing or w alking
4 E xtrem e bending of trunk preventing standing or w alking
L eg
0 N o dystonia present
1 Slight dystonia,but not causing im pairm ent; clinically insignificant
2 M ild dystonia.W alks briskly and unaided
3 M oderate dystonia.Severely im pairs w alking or requires assistance
4 Severe.U nable to stand or w alk on involved leg

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312 C .L. C O M E LLA E T A L.

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