296 TWSTRS
Summary
The trinucleotide repeat diseases are individually rare but
as a group are not uncommonly responsible for disabling
movement disorders. One of the major challenges of the
future is to develop specific treatments for these diseases.
See also: Aprataxin; Ataxia; Ataxia with Isolated
Vitamin E Deficiency; Ataxia-Telangiectasia; Ataxin;
ATM Gene; Atrophin-1; Cerebrotendinous Xanthoma-
tosis; Co-enzyme Q10; Fragile X-Associated Tremor/
Ataxia Syndrome (FXTAS); Friedreich’s Ataxia and Var-
iants; Huntington, George; Huntington’s Disease: Genet-
ics; Huntington’s Disease-like 2; Huntington’s Disease;
Idebenone and Friedreich Ataxia; Junctophilin; Refsum
Disease- a Disorder of Peroxisomal Alpha-oxidation;
SCA1; SCA2; SCA3, Machado–Joseph Disease; SCA4;
SCA5; SCA6; SCA7, Spinocerebellar Ataxia with Macular
Dystrophy; SCA8; SCA10; SCA11; SCA12; SCA13, 14, 15,
and 16; SCA17; Senataxin; Spinocerebellar Ataxia Type
19, 20, 21, 22, 23, 26; Spinocerebellar Ataxias Genetics;
Tocopherol Transfer Protein and Ataxia with Vitamin E
Deficiency; Westphal Variant.
Further Reading
Al-Mahdawi S, Pinto RM, Ismail O, et al. (2008) The Friedreich ataxia
GAA repeat expansion mutation induces comparable epigenetic
changes in human and transgenic mouse brain and heart tissues.
Human Molecular Genetics 17: 735–746.
Campuzano V, Montermini L, Molto MD, et al. (1996) Friedreich’s ataxia:
Autosomal recessive disease caused by an intronic GAA triplet
repeat expansion. Science 271: 1423–1427.
Everett CM and Wood NW (2004) Trinucleotide repeats and
neurodegenerative disease. Brain 127: 2385–2405.
Fogel BL and Perlman S (2006) An approach to the patient
with late-onset cerebellar ataxia. Nature Clinical Practice Neurology
2: 629–635.
TWSTRS
E Consky, Consultant Neurologist, Toronto, ON, Canada
ã 2010 Elsevier Ltd. All rights reserved.
Glossary
Anterior sagittal shift – Dystonic deviation of the
chin forward rather than downward.
Anterocollis – Dystonic deviation of the
chin downward in the sagittal plane toward
the chest.
Fogel BL and Perlman S (2007) Clinical features and molecular
genetics of autosomal recessive cerebellar ataxias. Lancet
Neurology 6: 245–257.
Gatchel JR and Zoghbi HY (2005) Diseases of unstable repeat
expansion: Mechanisms and common principles. Nature Reviews
Genetics 6: 743–755.
Greco CM, Berman RF, Martin RM, et al. (2006) Neuropathology of
fragile X-associated tremor/ataxia syndrome (FXTAS). Brain 129:
243–255.
Greene E, Mahishi L, Entezam A, Kumari D, and Usdin K (2007) Repeat-
induced epigenetic changes in intron 1 of the frataxin gene and its
consequences in Friedreich ataxia. Nucleic Acids Research 35:
3383–3390.
Hagerman PJ, Greco CM, and Hagerman RJ (2003) A cerebellar tremor/
ataxia syndrome among fragile X premutation carriers. Cytogenetic
and Genome Research 100: 206–212.
Harding AE (1981) Friedreich’s ataxia: A clinical and genetic study of 90
families with an analysis of early diagnostic criteria and intrafamilial
clustering of clinical features. Brain 104: 589–620.
Harding AE (1982) The clinical features and classification of the late
onset autosomal dominant cerebellar ataxias. A study of 11 families,
including descendants of the ‘the Drew family of Walworth’. Brain
105: 1–28.
Saveliev A, Everett C, Sharpe T, Webster Z, and Festenstein R (2003)
DNA triplet repeats mediate heterochromatin-protein-1-sensitive
variegated gene silencing. Nature 422: 909–913.
Schneider SA, Walker RH, and Bhatia KP (2007) The Huntington’s
disease-like syndromes: What to consider in patients with a negative
Huntington’s disease gene test. Nature Clinical Practice Neurology
3: 517–525.
The Huntington’s Disease Collaborative Research Group (1993) A novel
gene containing a trinucleotide repeat that is expanded and unstable
on Huntington’s disease chromosomes. Cell 72: 971–983.
Wild EJ, Mudanohwo EE, Sweeney MG, et al. (2008) Huntington’s
disease phenocopies are clinically and genetically heterogeneous.
Movement Disorders 23: 716–720.
Relevant Websites
http://www.ncbi.nlm.nih.gov/sites/entrez?db = omim – OMIM –
database of genetic disorders.
http://neuromuscular.wustl.edu/ – Neuromuscular home page –
database of genetic disorders.
http://www.geneclinics.org – Gene Tests – database with regular
reviews of genetic disorders.
Interrater reliability – The extent to which a rating
instrument yields reproducible, accurate, consistent,
and stable results when scored by different
observers.
Lateral shift – Horizontal displacement of the base
of the neck in the absence of tilting of the ear toward
the ipsilateral shoulder (unless accompanied by

laterocollis, which usually occurs in the opposite
direction).
Laterocollis – Dystonic tilting of the head laterally in
the coronal plane, moving the ear toward the
ipsilateral shoulder.
Posterior sagittal shift – Backward
displacement of the head without upward deviation
of the chin.
Retrocollis – Dystonic extension of the head,
producing upward excursion of the chin.
Rotational torticollis – Dystonic rotation of the nose
and chin around the longitudinal axis toward the
shoulder.
Sensory trick (gestes antagonistes) –
A pathognomonic feature of CD characterized by the
transient correction of head posture with the use of
maneuvers such as touching the face, neck, or head
with the hand or an object.
Tsui scale – An objective CD rating scale
developed by Tsui and colleagues,
evaluating the amplitude and duration of
sustained movements and head tremor and the
presence of shoulder elevation.
Validity – The extent to which a rating instrument
accurately measures what it is designed or purports
to measure. Content validity refers to the extent to
which a scale includes all relevant dimensions of the
condition being measured and whether it represents
these in reasonably weighted proportions. Construct
validity evaluates whether the rating instrument
measures what it intends to measure, and does not
measure what it is not intended to measure,
predictably distinguishing between groups and
producing results consistent with a predetermined
theoretical framework. Convergent validity refers to
the correlation with other measures of the same
construct or attribute. In addition, the responsiveness
of the rating scale to detect clinically significant
change is considered an aspect of validity.
TWSTRS
TWSTRS, the Toronto Western Spasmodic Torticollis
Rating Scale, developed by Consky and Lang in 1990, is a
multidimensional objective and subjective rating scale for
cervical dystonia (CD) with subscales for the relevant and
distinct clinical dimensions of CD: impairment severity,
associated disability and pain (see Figure 1). It has been
widely accepted and used as an outcome measure in thera-
peutic intervention studies, including Botulinum toxin
(BoNT) therapy, oral pharmacotherapy, and surgical trials
TWSTRS 297
for CD. It also has practical applicability in a clinical setting
in the ongoing management of individual CD patients. The
clinimetric properties of the TWSTRS have been defined
in a number of studies that have demonstrated TWSTRS
reliability, validity, and responsiveness to change following
treatment. A teaching tape for the TWSTRS severity scale
and a videotape protocol that contains the elements of a
standardized examination for CD have been developed to
promote consistent application.
The TWSTRS Severity Scale
The severity scale objectively quantifies the dynamic and
varied clinical spectrum of the involuntary movements or
abnormal postures of the head and neck seen in CD. The
maximum amplitude of excursion (A) (sustained or unsus-
tained) is determined on the basis of the standardized
examination, the patient being asked to allow the head
to deviate fully without resistance or the use of sensory
tricks, after activating and distracting maneuvers, walking
and sitting, and is determined for all dominant and minor
planes of head deviation: rotational torticollis (0–4), later-
ocollis (0–3), anterocollis or retrocollis (0–3), lateral shift
(0–1), and sagittal shift (0–1). The duration factor (B) (0–10)
for the dominant deviation quantifies the dynamic and
variable character of CD, which may change significantly
with posture and activity. To account for this variability,
the duration factor assesses first the proportion of time for
which there is any deviation from a neutral position and
second the proportion of time for which the amplitude of
the deviation is either predominantly maximal or sub-
maximal. The efficacy of sensory tricks (C) (0–2) is a reflec-
tion of CD severity. The effectiveness of sensory tricks
may vary considerably among patients, may wane over
time, and may change following therapeutic intervention.
Intermittent or sustained elevation or anterior displacement of
the shoulder (D) (0–3) is frequently present, commonly
ipsilateral to the direction of the turn or tilt. Examination
of the range of active motion (E) (0–4) in each of the three
axes rotational, lateral tilting, and flexion and extension,
without the aid of sensory tricks, is also determined. CD
severity is also quantified by determining the average time
(F) (0–4) on two attempts for whcih the patient is able to
maintain the head within 10 of a neutral position with
active resistance but without the use of sensory tricks. The
total TWSTRS Severity Scale score is a summation of
items A–F with a maximum score of 35.
The TWSTRS Disability Scale
Disability is task-specific and is not necessarily directly
proportional to the clinical severity of the abnormal postures
and movements of CD. The direction of head deviation,
coexisting dystonic involvement of other sites, pain,
298 TWSTRS

depression, sleep impairment, coping strategies, the avail-
ability of support, and the effectiveness of treatment may all
significantly influence the level of disability experienced by
an individual CD patient. The TWSTRS Disability Scale
consists of a broadly based assessment of the performance of
daily activities that may be affected by CD. General as well
as specific activity categories are assayed, including work
performance (employment or domestic work), activities of
daily living (hygiene, dressing, feeding), reading, television
viewing, driving, and leisure activities outside the home.
The extent to which social embarrassment rather than head
deviation or pain specifically contributes to disability was
initially included as an inverse item but was deleted follow-
ing initial testing of the scale. The maximum TWSTRS
Disability Scale score is 30.
The TWSTRS Pain Scale
Cervical pain is a frequent and prominent feature of CD
that often significantly contributes to disability and
impairment of quality of life. It is a separate and distinct
aspect of CD that may not be directly correlated with

The toronto western spasmodic torticollis rating scale (TWSTRS)

I. TWSTRS Severity Scale

A. Maximal excursion

Rate maximum amplitude of excursion asking patient not to oppose the

abnormal movement; examiner may use distracting or aggravating maneuvers.
When degree of deviation is between two scores, chose the higher of the two

1. Rotation (turn: right or left)

0 None
1 Slight (<1/4 range) (1–22°)
2 Mild (1/4–1/2 range) (23–45°)
3 Moderate (1/2–3/4 range) (46–67°)
4 Severe (>3/4 range) (68–90°)

2. Laterocollis (tilt: right or left) (exclude shoulder elevation)

0 None
1 Mild (1–15 ⬚)
2 Moderate (16–35 ⬚)
3 Severe (> 35 ⬚)

3. Anterocollis/retrocollis (a or b)
a) Anterocollis
0 None
1 Mild downward deviation of chin
2 Moderate downward deviation (approximates 1/2 possible range)
3 Severe (chin approximates chest)

b) Retrocollis
0 None
1 Mild backward deviation of vertex with upward deviation of chin
2 Moderate backward deviation (approximates '/z possible range)
3 Severe (approximates full range)

4. Lateral shift (right or left)

0 Absent
1 Present

5. Sagittal shift (forward or backward)

0 Absent
1 Present

Figure 1 (Continued)
 TWSTRS 299

the severity of motor impairment. The TWSTRS Pain
Scale includes an assessment of pain intensity, pain dura-
tion, as well as the affective components of pain result-
ing in disability. A weighted dimensional severity score
(0–10) for the patients usual, best, and worst pain, a
second-scale item which evaluates the duration of pain
typically experienced during the preceding week (0–5),
and a third-scale item which assesses the contribution of
pain to the disability (0–5) are scored with a maximum
TWSTRS Pain Scale score of 20.
The TWSTRS (total) score is the sum of the
TWSTRS Severity Scale score (0–35), the TWSTRS
Disability Scale score (0–30), and the TWSTRS Pain
Scale score (0–20) with a maximum score of 85.
Reliability and validity and comparative testing of
TWSTRS
Initial evaluation of TWSTRS by Consky, Lang, and col-
leagues showed substantial interobserver agreement for
each component of the TWSTRS Severity Scale, for the
whole Severity Scale, and for the TWSTRS Disability
and Pain Scales. The average severity component of the
TWSTRS Pain Scale was added subsequently and was not
included in the initial testing. High interrater reliability for
the change in patients’ scores prior to and 6 weeks following
BoNT treatment was demonstrated, indicating the respon-
siveness of the scales for objectively detecting clinical
change in severity. Convergent validity of TWSTRS was
evidenced by the high correlation of changes in TWSTRS

B. Duration factor

Provide an overall score estimated through the course of the standardized

examination after estimating maximal excursion (exclusive of asking patient to
allow head to deviate maximally). Weighted ⫻ 2 (see schematic representation
of scoring duration)

0 None
1 Occasional deviation (<25% of the time), most often submaximal
2 Occasional deviation (<25% of the time), often maximal or
intermittent deviation (25–50% of the time), most often submaximal
3 Intermittent deviation (25–50% of the time) often maximal or
frequent deviation (50–75% of the time), most often submaximal
4 Frequent deviation (50–75% of the time), often maximal or constant
deviation (>75% of the time), most of ten submaximal
5 Constant deviation (>75% of the time), often maximal

Schematic representation*
None 0

Submaximal 1
<25% of time (occasional)
Maximal 2

Submaximal 2
25–50% (intermittent)
Maximal 3

Submaximal 3
50–75% (frequent)
Maximal 4

Submaximal 4
75% (constant)
Maximal 5

*The rater determines the proportion of time that the dystonic head posturing is present
(left column) and then decides whether the deviations are most often maximal or
submaximal, having previously determined the maximal excursion score (A).

Figure 1 (Continued)
300 TWSTRS

Severity Scale scores from videotape raters with patients’
self-reported overall percent improvement. There was also
substantial agreement between changes in total TWSTRS
Severity Scale scores of videotape raters with changes in
patient self-reported TWSTRS Disability and Pain Scale
scores following BoNT treatment. The utility of the stan-
dardized TWSTRS Videotape Protocol was reflected by
the substantial agreement between blinded videotape raters
and a direct live examiner.
Further reliability testing of the TWSTRS severity
subscale was undertaken by Comella and colleagues in
the process of developing a teaching tape for scoring the
TWSTRS Severity Scale. There was statistically signifi-
cant interrater agreement for all individual components as
well as for the complete TWSTRS Severity Scale based
on the standardized videotape protocol.
Goertelmeyer and colleagues also demonstrated high
interrater reliability and high sensitivity to change follow-
ing BoNT as indicated by the mean change in TWSTRS
Severity score, and recently a study by Kaji and colleagues
confirmed the high interrater reliability of the TWSTRS
Severity Scale
Tarsy compared TWSTRS to the Tsui scale and to a
physician-rated subjective global improvement scale in
CD patients treated with BoNT. There was a significant
correlation between the posttreatment reduction of the
Tsui scale and total TWSTRS scores as well as the
TWSTRS Severity Scale scores. Both the TWSTRS
and the Tsui score reduction rates also correlated with
the global improvement scale. However, the TWSTRS
Disability and Severity Scale score reduction rates
showed a relatively weak correlation. TWSTRS Pain
Scale score reduction also showed a weak or no correla-
tion with the TWSTRS Severity scores or the Tsui scale
scores, with some patients experiencing a significant
reduction in pain scores despite the absence of objective
improvement in either the TWSTRS Severity score or
the Tsui scale score. The lack of correlation further

C. Effect of sensory tricks

0 Complete relief by one or more tricks

1 Partial or only limited relief by tricks
2 Little or no benefit from tricks

D. Shoulder elevation/anterior displacement

0 Absent
1 Mild (<1/3 possible range), intermittent or constant
2 Moderate (1/3–2/3 possible range) and constant (>75% of the time) or
severe (>2/3 possible range) and intermittent
3 Severe and constant

E. Range of motion (without aid of sensory tricks)

If limitation occurs in more than one plane of motion use individual score
that is highest

0 Able to move to extreme opposite position

1 Able to move head well past midline but not to extreme opposite
position
2 Able to move head barely past midline
3 Able to move head toward but not past midline
4 Barely able to move head beyond abnormal posture

F. Time (up to 60 s) for which patient is able to maintain head within 10° of
neutral position without the use of sensory ‘tricks’ (mean of two attempts)

0 0 > 60 s
1 46–60 s
2 31–45 s
3 16–30 s
4 <15 s

Total severity score = sum of A–F. Maximum score = 35

Figure 1 (Continued)
 TWSTRS 301

emphasizes that the clinical severity, disability, and pain
subscales of TWSTRS measure distinct and importantly
different attributes of CD.
Lindeboom and colleagues evaluated the extent to
which the therapeutic effectiveness of BoNT is captured
by various rating instruments. Changes in objective motor
impairment evaluated with the Tsui scale correlated
poorly with the TWSTRS Pain Scale, TWSTRS Disabil-
ity Scale, and handicap and quality-of-life (HRQoL) as
measured by subscales of the Medical Outcome Study
Scale. The decision to continue BoNT treatment in this
study correlated with a meaningful improvement to the
patient as reflected by changes in TWSTRS Disability
and HRQoL scale scores rather than changes in motor
severity Tsui scores. These findings further indicate that
each TWSTRS subscale assesses different aspects of the
disorder and its effect on patients.
Comella and colleagues investigated the internal con-
sistency of TWSTRS, determined the factor structure of
TWSTRS, and assessed whether the identified factors
form rational domains. High internal consistency was
demonstrated for the TWSTRS scale as a whole. Factor
structure analysis showed three clinically distinct factors:
a motor severity factor, a disability factor, and a pain
factor, again indicating that the severity, disability, and
pain subscales of TWSTRS form rational domains that
assess independent, different features of CD. Items found
to contribute least to the factor structure included the
effect of sensory tricks and the dichotomous items for
anterior and sagittal shift.
Grafe and Goertelmeyer undertook a study to demon-
strate the construct validity of the TWSTRS Severity
Scale and examine the responsiveness of the factorial
scores in a trial comparing different BoNT brands.

II. TWSTRS Disability Scale

1. Work (occupation or housework/home management)

0 No difficulty
1 Normal work expectations with satisfactory performance at usual
level of occupation but some interference by torticollis
2 Most activities unlimited, selected activities very difficult and
hampered but still possible with satisfactory performance
3 Working at lower than usual occupational level; most activities ham
peers, all possible but with less than satisfactory performance in
some activities
4 Unable to engage in voluntary or gainful employment; still able to
perform some domestic responsibilities satisfactorily
5 Marginal or no ability to perform domestic responsibilities

2. Activities of daily living

(e.g., feeding, dressing, hygiene, includes washing, shaving, makeup, etc.)
0 No difficulty with any activity
1 Activities unlimited but some interference by torticollis
2 Most activities unlimited, selected activities very difficult and
hampered but still possible using simple tricks
3 Most activities hampered or laborious but still possible; may use
extreme ‘tricks’
4 All activities impaired; some impossible or require assistance
5 Dependent on others in most self-care tasks

3. Driving
0 No difficulty (or has never driven a car)
1 Unlimited ability to drive but bothered by torticollis
2 Unlimited ability to drive but requires ‘tricks’ (including touching or
holding face, holding head against head rest) to control torticollis
3 Can drive only short distances
4 Usually cannot drive because of torticollis
5 Unable to drive and cannot ride in a car for long stretches as a
passenger because of torticollis

Figure 1 (Continued)
302 TWSTRS

Factorial analysis revealed a clinically meaningful four displacement; factor three by lateral shift and sensory
factor solution. Factor one was primarily loaded by rota- tricks; and factor four by retrocollis/anterocollis and sag-
tion, duration, range of movement, and time in midline; ittal shift. The total TWSTRS Severity Scale score as well
factor two by laterocollis and shoulder elevation/anterior as the factorial subscores was sensitive to change due to

4. Reading

0 No difficulty
1 Unlimited ability to read in normal seated position but bothered by
torticollis

2 Unlimited ability to read in normal seated position but requires use

of ‘tricks’ to control torticollis

3 Unlimited ability to read but requires extensive measures to control

torticollis or is able to read only in nonseated position (e.g., lying
down)

4 Limited ability to read because of torticollis despite tricks

5 Unable to read more than a few sentences because of torticollis

5. Television

0 No difficulty
1 Unlimited ability to watch television in normal seated position but
bothered by torticollis

2 Unlimited ability to watch television in normal seated position but

requires the use of tricks to control torticollis

3 Unlimited ability to watch television but requires extensive

measures to control torticollis or is able to view only in nonseated
position (e.g., lying down)

4 Limited ability to watch television because of torticollis

5 Unable to watch television for more than a few minutes because of
torticollis

6. Activities outside the home

(e.g., shopping, walking about, movies, dining, and other recreational activities)

0 No difficulty
1 Unlimited activities but bothered by torticollis
2 Unlimited activities but requires simple ‘tricks’ to accomplish
3 Accomplishes activities only when accompanied by others because
of torticollis

4 Limited activities outside home; certain activities impossible or

given up because of torticollis

5 Rarely if ever engages in activities outside the home

Total disability score = sum of 1–6. Maximum score = 30

Figure 1 (Continued)
 TWSTRS 303

the therapeutic intervention with the exception of factor
three (lateral shift and sensory tricks). The latter finding is
consistent with the study of Comella and colleagues,
which found a lack of variability of scale items for shift
and sensory tricks. There were no compensation effects
between the single items. The study’s findings provide
further support that the TWSTRS Severity Scale is a
valid and responsive tool for assessing change following
therapeutic intervention.
Several deficiencies and ambiguities of TWSTRS have
been identified by Comella and others, including the
absence of a scale component to assess the presence and
severity of associated dystonic head tremor, the lack of the
specification of midline and the full range of active
motion in each of the three planes, and the absence of
specification of the duration of relief provided by the use
of sensory tricks. Elimination or modification of the dichot-
omous items for lateral and sagittal shift as well as the item
for the effect of sensory tricks has been suggested because
of the lack of variability of these items. Further data-driven
refinement and testing of TWSTRS are required.
Substantial evidence for TWSTRS reliability and
validity as well as its responsiveness to detect a clinically
significant change following treatment has steadily accu-
mulated. TWSTRS encompasses the heterogeneity and
variability of the clinical features of CD, and the subscales
for severity, disability, and pain evaluate distinct aspects of
CD. Consistent application of TWSTRS is promoted by
the standardized examination contained in the TWSTRS
Videotape Protocol as well as the availability of a teaching

III. TWSTRS Pain Scale

1. Rate the severity of neck pain during the last week on a scale of 0–10 where a
score of 0 represents no pain and 10 represents the most excruciating pain
imaginable

Best 0–10
Worst 0–10
Usual 0–10

Severity = [(2 ⫻ usual) + best + worst]/4

Maximum score = 10

2. Rate the duration of neck pain

0 None
1 Present <10%' of the time
2 Present 10%–<25% of the time
3 Present 25%–<50% of the time
4 Present 50%–<75% of the time
5 Present >75% of the time

3. Rate the degree to which pain contributes to disability

0 No limitation or interference from pain
1 Pain is quite bothersome but not a source of disability
2 Pain definitely interferes with some tasks but is not a major contributor
to disability
3 Pain accounts for some (less than half) but not all disability
4 Pain is a major source of difficulty with activities; separate from this,
head pulling is also a source of some (less than half) disability
5 Pain is the major source of disability; without it most impaired activities
could be performed quite satisfactorily despite the head pulling

Total pain scale score = sum of 1–3. Maximum score = 20

TWSTRS score = Severity + disability + pain Maximum score = 85

Figure 1 (Continued)
304 TWSTRS

TWSTRS videotape protocol

1. Standing-viewed from front, side, and back x 10 s each

2. Walking-20 feet back and forth

a) Without instructions ⫻ 2
b) With instructions not to resist deviation (i.e., allow head to deviate
to maximum)⫻ 2

3. Sitting-in preferred or most comfortable position

a) Without instructions ⫻ 30 s
b) With instructions not to resist deviation ⫻ 30 s

4. Distracting or activating maneuvers: (sitting) (R and L separately)

each⫻ 10 s
a) Finger tapping
b) Opening and closing fist
c) Pronation and supination forearm
d) Arms outstretched, held under nose, finger to nose ⫻ 3
e) Foot tapping
5. Time in midline
With active resistance to deviation but without aid of sensory tricks or support.
Patient asked to maintain head in midline for as long as possible-maximum
60 s⫻ 2 attempts. Patient instructed not to talk.

6. Effect of ‘tricks’
Including touching side of face, holding chin, holding back of neck or head,
pressing against wall behind head, and other preferred tricks used by patient

7. Active range of movement (⫻ 2 each time)

Rotation, lateral flexion, forward flexion, extension

8. Writing name, sentence, repetitive words or phrase ⫻ 10 s

9. Lying supine ⫻ 20 s

Figure 1 The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS).

tape for the TWSTRS Severity Scale. TWSTRS has
gained widespread acceptance and is the outcome mea-
sure most commonly used in CD intervention studies.
See also: Botulinum Toxin; Cervical Dystonia; Dystonia;
Fahn–Marsden Rating Scale.
Further Reading
Comella CL, Fan W, Leurgans S, et al. (2005) Factor structure and
internal consistency of the Toronto Western Spasmodic Torticollis
Rating Scale (TWSTRS). Neurology 64(1): 129.
Comella CL, Stebbins GT, Goetz CG, et al. (1997) Teaching tape for the
motor section of the Toronto Western Spasmodic Torticollis Scale.
Movement Disorders 2: 570–575.
Consky ES (2006) Cervical Dystonia Rating Scales. In: Bouvier G, de
Soultrait F, and Molina-Negro P (eds.) Spasmodic Torticollis.
Clinical Aspects and Treatment, pp. 91–115. Paris: Expressions
Santé.
Consky ES, Basinki A, Belle L, et al. (1990) The Toronto Western
Spasmodic Torticollis Rating Scale (TWSTRS): Assessment of validity
and inter-rater reliability. Neurology 40(1): 445.
Consky ES and Lang AE (1994) Clinical Assessments of Patients
with Cervical Dystonia. In: Jankovic J and Hallett M (eds.)
Therapy with Botulinum Toxin, pp. 211–237. New York: Marcel
Dekker.
Goertelmeyer R and Grafe S (2006) Relationship between clinical
assessments of dystonia and treatment: A contribution to
pharmacosensitivity of the TWSTRS-severity scale. Movement
Disorders 21(15): 389–390.
Grafe S and Goertelmeyer R (2006) Factorial analysis of the Toronto
Western Spasmodic Torticollis Rating Scale (TWSTRS).
Neurotoxicity Research 9: 237.
Kaji R, Osawa M, and Yanagisawa N (2009) Inter-rater reliability
using the Toronto Western Spasmodic Torticollis Rating
Scale (TWSTRS) in patients with cervical dystonia. Brain Nerve
61: 65–71.
 TWSTRS 305

Lindeboom R, Brans JW, Aramideh M, et al. (1998) Treatment of Relevant Websites

cervical dystonia: A comparison of measures for outcome
assessment. Movement Disorders 13: 706–712.
Lindeboom R, de Haan RJ, Aramideh M, et al. (1996) Treatment www.mdvu.org/library/ratingscales/dystonia/ – TWSTRS rating scale,
outcomes in cervical dystonia: A clinimetric study. Movement TWSTRS examination and injection record.
Disorders 11: 371–376. www.movementdisorders.org/publications/audio_visuals.php –
Tarsy D (1997) Comparison of clinical rating scales in treatment of TWSTRS training videotape.
cervical dystonia with botulinum toxin. Movement Disorders 12:
100–102.