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
scale.T he w idely used Fahn-M arsden Scale (F-M )has notbeen
sufficiently tested across m ultiple centers and investigators.
T he D ystonia Study G roup developed the U nified D ystonia
R 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. In
this study,25 dystonia experts evaluated the U D R S,F-M ,and
G D S for internal consistency and reliability. O ne hundred
dystonia patients w ere videotaped using a standardized video-
tape protocol. E ach exam iner rated 20 patients using the
U D R S, F-M , and G D S in random order. T he exam iner then
assessed each scale for ease of use. Statistical analysis used
C ronbach’s !,intraclass correlation coefficients (IC C ),gener-
alized w eighted " statistic, and K endall’s coefficient of con-
D ystonia is defined as a syndrom e consisting ofinvol-
untary m ovem ents characterized by tw isting orsustained
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 first dystonia
scale evaluated for its clinom etric properties.In a study
using 10 dystonia patients and 4 raters, the reliability,
inter-rateragreem ent,and concurrentvalidity ofthe 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
† 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
Received 5 July 2002; Revised 29 August 2002; Accepted 29 August
2002
303
cordance.T he U D R S,F-M ,and G D S show ed excellentinternal
consistency (C ronbach’s ! 0.89 – 0.93) and good to excellent
correlation am ong the raters (IC C range from 0.71– 0.78).Inter-
rater agreem ent w as fair to excellent (K endall’s 0.54 – 0.87; "
0.37– 0.91) being low est for eyes, jaw , face, and larynx. T he
m 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 severity
ratings.A m ong scales,the totalscores correlated (Pearson’s r,
0.977– 0.983).O verall,74% ofraters found the G D S the easiest
to apply.T he G D S w ith its sim plicity and ease of application
m ay be the m ost useful dystonia rating scale. © 2002 M ove-
m ent D isorder Society
K ey w ords: dystonia;rating scale;m ovem entdisorder;out-
com e assessm ent
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 nothave rep-
resented the fullspectrum ofdystonia severity thatw ould
be encountered in a m ulticenter study. Som e of the
lim itations in the F-M include the variable definition of
body areas,and a w eighting factor of 0.5 thathalves 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 SG 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
304 C .L. C O M E LLA E T A L.
validated w ithin the T oronto W estern Spasm odic T orti-
collis Scale (T W ST R S).4 – 6 In contrast to the F-M , the
U D R S has no w eighting factors forany body region.T he
G D S is a globalscale applied to individualbody regions.
In this D SG -initiated study,the specific aim s w ere to
evaluate the internal consistency, inter-rater reliability
and clinical applicability of the F-M , U D R S, and G D S
across m ultiple sites in a large num ber of dystonia pa-
tients encom passing the full spectrum of dystonia
severity.
P A T IE N T S A N D M E T H O D S
P atients
Patients w ere included in the study ifthey had prim ary
dystonia and w ere follow ed in the outpatientM ovem ent
D isorders clinic offices at R ush-Presbyterian-St.L uke’s
M edical C enter. Inform ed consent, as approved by the
Institutional R eview B oard at R ush Presbyterian St.
L uke’s M edical C enter, w as obtained from all partici-
pants. T he diagnosis of prim ary dystonia w as based on
presence ofdystonia and absence ofadditionalneurolog-
ical signs or causes for dystonia.In particular,attention
w as paid to recruiting patients w ith generalized dystonia
so that each m aster tape could have adequate represen-
tation of types and range of dystonia.
Investigators
T he 25 rating investigators from 20 institutions are
listed in A ppendix 1. T he Principal Investigator (C L C )
w as notincluded as a rating investigator,and carried out
the initial screening of the videotapes of all patients.
T here w ere no investigators from the recruiting institu-
tion to prevent any rater from having previous know l-
edge ofthe patients included forrating.E ach investigator
w as a specialistin M ovem entD isorders w ith expertise in
the evaluation of dystonia.
V ideotaping P rotocol and D evelopm ent
of R ating T apes
T here w ere 103 patients videotaped using a standard
videotape protocol that incorporates and expands the
videotape protocolincluded w ith the F-M (see A ppendix
2).3 T he videotape protocolincludes exam ination ofeach
body region at rest and during activation procedures.
Patients w ere videotaped in a uniform m anner. A ll the
videotapes w ere evaluated by the PI (C L C ) w ho rated
each of 10 body areas for severity of dystonia using a 0
to 10 scale, w ith 0 defined as no dystonia and 10 as
severe dystonia.T hese scores w ere used to allocate pa-
tients to a severity level and then random ly allocate
patients such thateach m asterevaluation tape included a
M ovem ent D isorders,V ol.18,N o.3,2003
range of dystonia severity. E ach investigator rated tw o
m aster evaluation tapes w ith 10 patients included on
each tape,or a totalof 20 patients.N o pair of investiga-
tors rated the sam e tw o tapes. A statistician (SL ) used
com puter-generated random num bers to allocate pairs of
tapes to the raters. T he rating investigator view ed the
evaluation videotapes a total of three tim es using the
U D R S for rating during one view ing, the G D S during
another view ing and the F-M during another view ing.
T he order of scale application w as random ized.
R ating Scales
T he U D R S includes ratings for 14 body areas includ-
ing eyes and upper face, low er face, jaw and tongue,
larynx, neck, trunk, shoulder/proxim al arm (right and
left),distalarm /hand (rightand left),proxim alleg (right
and left),and distalleg/foot(rightand left)(A ppendix 3).
For each of the 14 body areas assessed,the U D R S has a
severity and a duration rating. T he severity rating is
specific foreach body region assessed and ranges from 0
(no dystonia) to 4 (extrem e dystonia).T he duration rat-
ing is m odified from the duration factorofthe T W ST R S,
and ranges from 0 to 4. T he D uration rating assesses
w hether dystonia occurs at rest or w ith action, and
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
severity and duration factors.T he m axim altotalscore of
the U D R S is 112.
T he F-M rating scale (see A ppendix 4) evaluates dys-
tonia in nine body areas,including eyes,m outh,speech
and sw allow ing,neck,trunk,and rightand leftarm and
leg.T he arm s and legs are given one rating each,w ithout
distinguishing proxim aland distalelem ents.For each of
the nine body regions,severity ratings range from 0 (no
dystonia) to 4 (severe dystonia). T he provoking factor
rating assesses the situation under w hich the dystonia
occurs and ranges from 0 (no dystonia) to 4 (dystonia at
rest).T he score for the eyes,m outh,and neck are each
m ultiplied by 0.5 before being entered into the calcula-
tion of the total score.T he total score of the F-M is the
sum of the products of the provoking, severity and
w eighting factors.T he m axim altotalscore on the F-M is
120.2
T he G D S rates dystonia severity in the 14 body areas
already described for the U D R S (see A ppendix 5).T he
G D S is a L ikert type scale w ith ratings from 0 to 10 (0
is no dystonia, 1 m inim al, 5 m oderate and 10 severe
dystonia) (A ppendix 4).T here are no m odifying ratings
or w eighting factors in the G D S. T he total score is the
sum of the scores for all the body areas. T he m axim al
total score of the G D S is 140.
 R A TIN G SC A LE S F O R D YSTO N IA
A fter ratings w ere com pleted using all three scales,
each investigator com pleted a standard questionnaire for
each scale thatassessed the investigator’s opinion ofease
of application,usefulness in an office setting and useful-
ness in m ulticenter trials.
Statistical A nalysis
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
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 foreach patient.Sum m ary statistics
ofthe overallscores are presented as m ean # SD ratings
and range,Pearson’s correlation w ere used for pair-w ise
com parison of the total scores of the three scales.
T he internalconsistency of each scale w as assessed
by C ronbach’s !. O verall inter-rater agreem ent w as
assessed using intraclass correlation coefficient(IC C ).
T he IC C w as first 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 entforbody regions w as analyzed in
tw o w ays:K endall’s coefficientofconcordance 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 endall’s coefficient of concordance provides a
m easure of the consistency am ong raters in the rankings
of dystonia severity. K endall’s coefficient of concor-
dance for each body region w as com puted for each 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 –1,2–3,4 – 6,7–10; 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 endall’s 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.
305
T A B L E 1. Sum m ary,internal consistency,and intraclass
correlation coefficients of overall dystonia ratings for each
rating scale
U D R Sa F-M b
GDS
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
Intraclass correlation
coefficient 0.71 0.78 0.72
a
T en subjects had only 4 (instead of 5) ratings.
b
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;
G D S,G lobal D ystonia R ating Scale.
R E SU 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 other
neurological conditions besides prim ary dystonia. F-M
data on 2 subjects from one rater and U D R S data on 8
subjects from anotherraterw 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 ofdystonia 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 ofthe three scalesw asfound to have a high levelof
internalconsistency,w ith C ronbach’s !ranging from 0.89
to 0.93 (Table 1).C ronbach’s!is a function ofthe num ber
ofitem s on a rating scale and inter-ratercorrelation;itis an
index ofhow stable and consistentthe item son 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).The results of
the K endall’s coefficientofconcordance foreach body area
foreach scale are show n in Table 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 .The agree-
m ent is low est for the larynx and speech for the U D R S
(K endall’s $ 0.56) and for the G D S (K endall’s $ 0.59).
U pper face and eyes show ed the low estagreem enton the
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
in each body area isshow n in Table 3.In general,the m otor
severity ratings ofboth the U D R S and F-M show ed greater
agreem entthan the duration or provoking factors.A s seen
previously w ith the K endall’s,agreem entam ong raters w as
low est for upper face and eyes (m otor severity of U D R S
"$ 0.52;F-M "$ 0.52;G D S "$ 0.58).
P airw ise C om parisons and P earson’s C orrelations
T he total scores for the three scales are highly corre-
lated w ith each other.T he three pairw ise scatterplots are
show n in Figure 1.T he scales had Pearson’s correlations
of0.983 (U D R S and G D S),0.977 (F-M and U D R S),and
0.980 (F-M and U D R S).
Investigator Q uestionnaires
T he results ofthe investigatorquestionnaire are show n
in T able 4.Seventy-four percentof the rating investiga-
tors found the G D S extrem ely or very easy to apply and
82% found itusefulform easuring dystonia severity in an
office setting.In contrast,only 5% ofinvestigators found
the U D R S easy to use,and 38% the F-M .
D ISC U SSIO N
T he m easurem entof dystonia severity lies w ith clini-
cal exam ination and the developm ent of reliable and
valid rating scales. T he testing of new therapeutic ap-
proaches to dystonia, including surgical interventions,
w ill require collaboration am ong m ultiple investigators
and study sites. T o im plem ent m ulticenter studies, a
reliable and valid instrum entthatcan assess the spectrum
of dystonia severity is necessary.9 T he F-M dystonia
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-
strated reliability and validity of the F-M .3 W hether this
rating scale is usefulforlarge clinicaltrials had notbeen
assessed until the current study.
T he U D R S w as developed to address the perceived
lim itations of the F-M . T he U D R S divides the body
regions into sm aller m ore defined areas, adding a new
m odifying rating scale that w as successfully used for
focaldystonia (T W ST R S),4 – 6 and elim inates the w eight-
ing factor that lessened the contribution of dystonia se-
verity in certain body regions in the F-M scale.T he G D S
w as designed as a sim ple direct assessm ent of overall
severity of dystonia in each body area. D espite differ-
ences in scale construction, the m easures of internal
consistency and inter-raterreliability w ere w ithin accept-
able range forallthree scales.T he C ronbach’s !m ay be
som ew hatinflated because ofthe num berofitem s w ithin
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
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.
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-
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
research sites. A s w ith rating scale developm ent in other
m ovem entdisorders,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 for
validity and responsivity to change are also necessary to
understand the clinical utility of the scales. The parallel
developm ent of a teaching tape that dem onstrates the rat-
ingsforeach body area,especially face and eyes,w illincrease
the inter-rateragreem entfordystonia in these areas.
A cknow ledgm ent:T his w ork w as supported by a grantfrom
the D ystonia M edical R esearch Foundation.
A P P E N D IX 1
The follow ing D ystonia Study G roup sites and investigators partici-
pated in this study:
M .Stacy,B arrow N eurologicalInstitute,Phoenix,A Z ;D .T arsy,
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-
rologicalInstitute,E nglew ood,C O ;B .Ford,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 ospital,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 ondon, O ntario; M .F. G ordon, L ong Is-
land Jew ish H ospital,N ew H yde Park,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-
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
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-
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 Southern 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 P P E N D IX 2. D ystonia Study G roup V ideotape exam ination protocol

A rea assessed
Part 1: E yes and upper face
Part 2: L ow er face,jaw ,
tongue,larynx
Part 3: N eck
Part 4: Shoulders and upper
arm s,distal arm and
hands
Perspective
C lose view of head and shoulders; sitting
unsupported in chair w ithout back
Patient seated
Sw allow interview
Seated in chair,close view head and shoulders
Far view of upper half of body
A ctivity
A t rest (10 sec)
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)
C lose view of face at rest (10 sec)
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)
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?
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)
A rm s extended supinated: 5 sec
A rm s extended pronated: 5 sec
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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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

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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.
A P P E N D IX 5
T he G lobal D ystonia Severity R ating Scale
T he globalscore is an overallscore for the body area.T he investi-
gator rates the patient in relationship to all patients. If the dystonia
changes during the exam ination,the rating for the m axim aldystonia is
recorded.
E ach body area is rated from 0 to 10:
0 N o dystonia present in that body area
1 M inim al dystonia
5 M oderate dystonia
10 M ost severe 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) distal leg and foot,and 10) trunk.
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