Title:

Test–retest reliability of the Dyskinesia Impairment Scale: measuring

dystonia and choreoathetosis in dyskinetic cerebral palsy

Authors
Inti Vanmechelen PhD1, Bernard Dan, MD2,3, Hilde Feys, PT4, Elegast Monbaliu, PT1,5
1
Department of Rehabilitation Sciences, KU Leuven, Campus Brugge, Brugge. 2Department
of Neurology, Université Libre de Bruxelles, Brussels, Belgium; Inkendaal
Rehabilitation Hospital, Vlezenbeek, Belgium.
3
Faculty of Medicine, Université libre de Bruxelles, Brussels, Belgium.
4
Department of Rehabilitation Sciences, KU Leuven, Leuven.
5 Sint Jozef Instituut, Antwerpen, Belgium

Corresponding author: I. Vanmechelen. Department of Rehabilitation Sciences, KU

Leuven, Campus Brugge. Spoorwegstraat 12, 8200 Bruges. E-mail:
inti.vanmechelen@kuleuven.be

1
Abstract
Aim The aim of this study was to assess test-retest reliability of the Dyskinesia Impairment
Scale (DIS) in children and young adults with dyskinetic cerebral palsy.
Method Dystonia and choreoathetosis was assessed in 15 participants with dyskinetic cerebral
palsy (13 males; 5-22y, mean 14y, SD 5y) using the Dyskinesia Impairment Scale in two
separate sessions over seven days. Exclusion criteria were changes in muscle relaxant
medication within the previous 3 months, orthopaedic or neurosurgical interventions within
the previous year, and spinal fusion. Intra-class correlation coefficient (ICC), confidence
intervals (CI), standard error of measurement, and the minimal detectable difference were
determined for test-retest reliability.
Result ICCs of the DIS, the dystonia subscale and the choreoathetosis subscale of the DIS
were 0.98 (95% CI=0.94-0.99), 0.97 (95% CI=0.92-0.99) and 0.96 (95% CI=0.90-0.99). The
standard error of measurement and minimal detectable difference were 2.6% and 7.2%.
Interpretation The DIS is a reliable tool to assess dystonia and choreoathetosis; it remains
stable over time in children and young adults with dyskinetic cerebral palsy. These results add
to the current evidence for good clinimetric properties of the DIS.

What this paper adds:

 Dyskinesia impairment scale (DIS) shows stability in scoring in the clinical
assessment of dystonia and choreoathetosis in individuals with dyskinetic cerebral
palsy.
 The total score of the DIS, and both the dystonia and choreoathetosis subscales are
clinically useful.

Running foot title:

Test –retest reliability of the Dyskinesia Impairment Scale

Key words
Dyskinetic Cerebral Palsy
Dystonia
Choreoathetosis
Dyskinesia Impairment Scale
Test-retest reliability

2
Cerebral palsy (CP) comprises a group of developmental disorders of movement and posture
and is the most common cause of severe physical disability in children, occurring in 1.7-3.1
per 1000 live births.1, 2 CP can be subdivided into three categories based on their dominant
motor abnormalities: spastic, ataxic and dyskinetic CP (DCP). 3 DCP is the second most
prevalent type of CP.4 Dystonia and choreoathetosis are the dominant movement disorders in
this type. Dystonia is characterized by abnormal postures, involuntary twisting and repetitive
movements due to sustained or intermittent muscle contractions. Choreoathetosis is
dominated by rapid, involuntary, jerky and fragmented movements (chorea) and writhing or
contorting movements (athetosis).5 Both dystonia and choreoathetosis impact heavily on
activities of daily living and independence, and associated disorders are frequent.6-8

Dystonia and choreoathetosis occur co-concurrently in DCP and can be identified as distinct
from each other, often with more severe dystonia in comparison with choreoathetosis. 9 The
clinical presentation of dystonia and choreoathetosis is known to vary over time, dystonia
being aggravated by non-specific stimuli such as emotion and stress.4 The fluctuating
presentation of symptoms in DCP complicates the assessment of dystonia and
choreoathetosis, since their variable appearance challenges the grading of these movement
disorders in a reliable way. The recognition of dystonia and choreoatheosis is still perceived
as ‘difficult’ in clinical practice. 10 A more reliable and easy to handle measurement tool can
contribute to a more robust diagnosis, with consequences for treatment options and outcome
evaluation.

Several clinical measurement scales to assess dystonia have been developed over the years.
The Barry-Albright Dystonia Scale (BADS) has been widely used for dystonia in CP but
concurrent validity has not been assessed.11-13 The Burke-Fahn-Marsden Dystonia Rating
Scale (BFMDRS) and the Unified Dystonia Rating Scale (UDRS) were developed to assess
dystonia in primary dystonia and not validated in CP.14, 15 Studies validating the psychometric
properties of these scales are scarce, especially when focusing on the assessment of dystonia
over time.11 Standard error of measurement and minimal detectable difference – a concept
designed for the assessment of change – have not been reported by any of the authors of the
previously mentioned scales. Given the fluctuating patterns of dystonia and choreoathetosis
and their impact on activities of daily living, the importance of assessing the effect of
(medicinal) intervention on the severity of dystonia and choreoathetosis is an indispensable
part of a reliable measurement tool. A reliable assessment of the effect of intervention – both
in dystonia and choreoathetosis – can optimize the individual treatment management plan of
each unique patient, adapted to their specific needs and desires.16

The Dyskinesia Impairment Scale (DIS) was developed to provide a reliable assessment tool
for both dystonia and choreoathetosis in DCP specifically. 5 It includes a dystonia subscale
(DIS-D) and choreoathetosis subscale (DIS-CA) that score the presence and severity of these
movement disorders in various body regions at rest and during activity. 5 The DIS is listed as
practical tool by the Dystonia Care Pathway of the American Academy of Cerebral Palsy and
Developmental Medicine.17 It showed high internal consistency and interrater reliability, but
its test-retest reliability has not been previously assessed.11

The aim of this study is to examine the reliability of measuring dystonia and choreoathetosis
with the DIS between separate test sessions in children and young adults with DCP. The
hypothesis is that the DIS is a reliable tool to assess dystonia and choreoathetosis over time.

3
Methods
Participants
Participants were recruited from special education schools for children with motor disability.
The participants are a subset of the participants included in the study of Monbaliu et al. 5 who
were available for a re-test recording. Individual patient characteristics are presented in
Appendix I. Inclusion criteria were predominant dyskinetic CP diagnosed by a paediatric
neurologist within a reference centre for CP and birth at term. Exclusion criteria were changes
in muscle tone-changing medication within the last three months, orthopaedic or
neurosurgical interventions within the last year, and spine fusion. Ethical approval was
obtained from the Ethical Committee of the KU Leuven. All participants and their parents
provided written informed consent.

Procedure
Participants were videotaped according to the DIS video protocol, which contains three rest
postures and 24 activities. During filming, participants wore a short for boys and a short and a
bra for girls to maximize visibility of all body parts for scoring. The DIS contains two
subscales, the DIS-D and the DIS-CA, both evaluating amplitude and duration of respectively
dystonia and choreoathetosis in 12 body regions during action and rest. To assess reliability
over time, participants were videotaped twice, the second time within seven days after the
first recording in order to minimize environmental or therapy effects. Test and re-test videos
were filmed by the same assessor (EM). Scoring of both test and re-test videos was done by
the same rater (EM) within 15 days, with three months in between the scoring of the test and
re-test video to minimize recall bias. The DIS film protocol varies between 40 and 60
minutes. Scoring was done on a 5-point scale from 0 to 4 for each body part, both during
action and rest. Each score was summed for a total dystonia score, a total choreoathetosis
score and a total DIS score. To assess test-retest reliability for the region scores, all scores for
each region both in activity and rest, were summed.

Statistical methods
Shapiro-Wilk test was used to explore whether the data was distributed normally. Levene’s
test was used to asses homogeneity of variances between test and retest.
Intraclass correlation coefficients (ICC) and 95% confidence intervals (CI) were used for total
scores and item scores of the DIS. ICC model (2,1) was applied, using the same set of raters
randomly selected from a group, with the purpose of generalizing the results beyond this
study.18 ICCs higher than 0.90 were considered as excellent and between 0.75 and 0.90 as
good. We considered ICC values between 0.60 and 0.75 as moderate and less than 0.60 as
poor.18 Standard error of measurement (SEM) and minimal detectable difference (MDD) were
calculated with the formula SEM=SDx√(1-ICC) and SDD=SEMx1.96x√2. All statistics were
calculated with SPSS 16.0 (SPSS Inc, Chicago IL, USA) with 0.05 as significance level.

Results
Fifteen participants with DCP (5-22 years, mean 14 years, SD 5 years) were included for test-
and retest assessment over seven days. Shapiro–Wilk test indicated that all but eight of the 34
items for test and retest of the DIS were normally distributed. Of those eight, four tended
towards significance with p-values between 0.04 and 0.05. Analysis of variance revealed
homogeneity of variances between groups for all test items. Test-retest reliability was
excellent for the total score of the DIS, the DIS-D and the DIS-CA with ICCs of 0.98 (95%
CI=0.94-0.99), 0.97 (95% CI=0.92-0.99) and 0.96 (95% CI=0.90-0.99). ICCs, 95% CI and p-
values of the total subscale scores and region scores are presented in Table I.

4
[Table 1 near here]

For the DIS-D, total scores of duration, amplitude and sum of the two ranged from 0.95 to
0.97 during action and from 0.90 to 0.93 during rest. All regions showed moderate to
excellent reliability during action except for the right proximal arm. During rest, the duration
factor for mouth and neck and the amplitude factor for neck and left proximal arm showed
lower reliability. During action, all p-values were significant except for right proximal arm
(0.129) and mouth (0.138) amplitude. During rest, only the neck amplitude (0.154) was not
significant. For the summation of duration and amplitude, arm right proximal during action
(0.053) was not significant.

For the DIS-CA, ICCs of the total scores of duration, amplitude and summation of both
ranged from 0.90 to 0.95 during action and 0.90 to 0.93 during rest. Regions scores during
action showed moderate to excellent reliability except for the eyes for duration factor and the
eyes, trunk and right distal arm for amplitude factor. During rest, reliability coefficients were
moderate to good, except for the left proximal arm amplitude. During action, all p-values
were significant except the right distal arm amplitude factor (0.063). During rest, all p-values
were significant except the right proximal arm (0.279) and trunk (0.071) amplitude factor.

The SEM and MDD values for the total DIS were 2.6% and 7.2% respectively. The SEM and
MDD were 3.7% and 10% for the DIS-D and 3.6% and 9.9% for the DIS-CA.

Discussion
Overall, test-retest reliability scores were excellent for both the DIS and the dystonia and
choreoathetosis subscales. The region scores of the subscales showed generally moderate to
excellent test-retest reliability during action and during rest. For the dystonia subscale, ICCs
of 0.27 and 0.36 were found for the neck region during rest and the amplitude of the arm right
proximal region during action respectively. In-depth analysis revealed some larger differences
in scores between test and retest which may have led to lower reliability.18

A few choreoathetosis regions showed low ICC scores. These results can be understood in
view of the fluctuating nature of both dystonia and choreoathetosis. The amplitude factor for
the left proximal arm during rest had a very low ICC. Further data exploration showed a
limited variability of the scores for this region. It is well known that ICC is sensitive for
limitations in variability. This may have contributed to a lower reliability.18 Nevertheless,
overall test-retest reliability was very high, which allows the implementation of the scale over
different time periods.

The SEM and MDD showed small values. The MDD was 7.2% for the total DIS, 10% for the
dystonia subscale and 9.9% for the choreoathetosis subscale. In clinical use this means that a
score difference of respectively 10% to 15% for the dystonia subscale and less than 10% for
the choreoathetosis subscale and total DIS is necessary to be reasonably sure that effective
improvement has occurred, rather than the difference being due to measurement errors. 18
These scores are in line with, but lower than the previously stated MDD values of the
measurement scales mentioned above (BADS, BFMDRS, UDRS), which were based on
interrater reliability values rather than test-retest values.12-15 Comparison with MDD values of
test-retest measurements is not possible, as these are not available for other measurement
scales in the current literature. The current MDD values are in a clinically acceptable range to
assess changes or improvements after an intervention, which is an important characteristic of

5
a reliable measurement tool. In other studies, MDD values for other measurement scales e.g.
for upper limb function in children with CP have varied between 9% and 13%. 19 Low MDD
values, as presented for the DIS, will benefit sensitivity. Additionally, these low MDD values
support the use of the scale in long-term follow-up and intervention studies. A recent ITB-
responsiveness study is consistent with our results.20

Although the lengthy film protocol may be considered less user-friendly, the DIS does
comprise the assessment of both dystonia and choreoathetosis, offering an overall picture of
the presence and severity of dystonia and choreoathetosis. Another feature of the thorough
assessment of the DIS is the time it takes to score the participants, which is not readily
available in the clinical setting. For this reason, analysis techniques to shorten the DIS are
currently being explored and responsiveness studies to aim for item reduction of the scale are
ongoing.

In conclusion, the good results of the assessment of dystonia and choreoathetosis over time
show that the DIS can be used in clinical practice and research to assess the effect of various
interventions on the presence and severity of dystonia and choreoathetosis.

Funding

This work was supported by a grant of the Marguerite-Marie Delacroix Foundation.

6
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 TABLES
Table I: Test-retest reliability of the Dyskinesia Impairment Scale.
act rest
Dystonia Duration Amplitude ∑(D+A) Duration Amplitude ∑(D+A)
Subscale
ICC 95%CI p-value ICC 95%CI p-value ICC 95%CI p-value ICC 95%CI p-value ICC 95%CI p-value ICC 95%CI p-value

1 Eyes 0.86 0.62-0.95 0.001 0.78 0.47-0.92 0.85 0,61-0.95 <0.001   0.94 0.83-0.98 <0.001 0.84 0.59-0.94 <0.001 0.94 0.84-0.98 <0.001
0.002
2 Mouth 0.89 0.69-0.96 <0.001 0.29 -0.238-0.69 0.94 0.82-0.98 <0.001 0.55 0.07-0.82 0.014 0.72 0.35-0.90 0.001 0.67 0.25-0.87 0.002
0.138
3 Neck 0.77 0.45-0.92 <0.001 0.79 0.48-0.92 0.83 0.57-0.94 <0.001 0.55 0.42-0.91 <0.001 0.27 -0.26-0.68 0.154 0.49 -0.14-0.79 0.012
<0.001
4 Trunk 0.95 0.87-0.98 <0.001 0.95 0.87-0.99 0.96 0.89-0.99 <0.001 0.83 0.56-0.94 <0.001 0.72 0.35-0.90 0.001 0.82 0.54-0.94 <0.001
<0.001
5 Arm RP 0.51 0.02-0.80 0.024 0.36 -0.17-0.73 0.53 0.05-0.81 0.053 0.93 0.80-0.98 <0.001 0.86 0.63-0.95 <0.001 0.86 0.63-0.95 <0.001
0.129
6 Arm LP 0.89 0.72-0.96 <0.001 0.73 0.37-0.90 0.84 0.58-0.94 <0.001 0.87 0.65-0.95 <0.001 0.62 0.17-0.85 0.006 0.74 0.39-0.91 <0.001
<0.001
7 Arm RD 0.99 0.97-1.00 <0.001 0.96 0.90-0.99 0.98 0.95-0.99 <0.001 0.61 -0.03-0.69 0.031 0.68 0.27-0.88 0.002 0.68 0.28-0.88 0.006
<0.001
8 Arm LD 0.99 0.98-1.00 <0.001 0.96 0.88-0.99 0.99 0.96-1.00 <0.001 0.84 0.59-0.94 <0.001 0.80 0.51-0.93 <0.001 0.85 0.62-0.95 <0.001
<0.001
9 Leg RP 0.85 0.61-0.95 <0.001 0.89 0.72-0.96 0.89 0.70-0.96 <0.001 0.94 0.83-0.98 <0.001 0.86 0.63-0.95 <0.001 0.91 0.75-0.97 <0.001
<0.001
10 Leg LP 0.88 0.68-0.96 <0.001 0.92 0.77-0.97 0.90 0.72-0.96 <0.001 0.98 0.93-0.99 <0.001 0.88 0.68-0.96 <0.001 0.95 0.84-0.98 <0.001
<0.001
11 Leg RD 0.73 0.36-0.90 0.001 0.82 0.54-0.94 0.78 0.45-0.92 <0.001 0.69 0.30-0.88 0.002 0.73 0.36-0.90 0.001 0.73 0.36-0.90 0.001
<0.001
12 Leg LD 0.80 0.51-0.93 <0.000 0.85 0.60-0.95 0.91 0.76-0.97 <0.001 0.60 0.14-0.84 0.007 0.73 0.37-0.90 0.001 0.67 0.25-0.87 0.002
<0.001

Total score 0.96 0.90-0.99 <0.001 0.95 0.87-0.99 0.97 0.92-0.99 <0.001 0.92 0.78-0.97 <0.001 0.90 0.75-0.97 <0.001 0.93 0.80-0.98 <0.001
<0.001

∑(D+A), summation of duration and amplitude factor ; RP, right proximal ; LP, left proximal ; RD, right distal ; LD, left distal

1
 Table I (continued)
act rest
Choreoathetosis Duration Amplitude ∑(D+A) Duration Amplitude ∑(D+A)
Subscale
ICC 95%CI p-value ICC 95%CI p-value ICC 95%CI p-value ICC 95%CI p-value ICC 95%CI p-value ICC 95%CI p-value

1 Eyes 0.57 0.10-0.88 0.011 0.47 -0.26-0.79 0.55 0.07-0.-82 0.013   0.74 0.38-0.90 0.001 0.78 0.45-0.92 <0.001 0.76 0.42-0.91 <0.001
0.033
2 Mouth 0.72 0.36-0.89 0.001 0.84 0.59-0.94 0.85 0.61-0.95 <0.001 0.92 0.79-0.97 <0.001 0.50 0.01-0.80 0.025 0.77 0.44-0.92 <0.001
0.000
3 Neck 0.78 0.45-0.92 <0.001 0.69 0.30-0..89 0.77 0.44-0.92 <0.001 0.85 0.60-0.95 <0.001 0.81 0.53-0.93 <0.001 0.84 0.59-0.94 <0.001
0.002
4 Trunk 0.87 0.65-0.95 <0.001 0.51 0.01-0.81 0.79 0.48-0.92 <0.001 0.70 0.32-0.89 0.001 0.36 -0.14-0.74 0.071 0.63 0.19-0.86 0.004
0.025
5 Arm RP 0.83 0.57-0.94 <0.001 0.77 0.44-0.92 0.83 0.56-0.94 <0.001 0.70 0.32-0.89 0.001 0.66 0.25-0.87 0.003 0.69 0.30-0.89 0.001
<0.001
6 Arm LP 0.93 0.88-0.96 <0.001 0.95 0.85-0.98 0.96 0.89-0.99 <0.001 0.69 0.30-0.88 0.002 0.16 -0.37-0.61 0.279 0.54 0.06-0.82 0.015
<0.001
7 Arm RD 0.60 0.15-0.85 0.004 0.41 -0.13-0.76 0.55 0.06-0.82 0.016 0.88 0.69-0.96 <0.001 0.63 0.20-0.86 0.004 0.78 0.45-0.92 <0.001
0.063
8 Arm LD 0.91 0.78-0.97 <0.001 0.69 0.29-0.88 0.87 0.66-0.96 <0.001 0.74 0.38-0.90 0.001 0.64 0.21-0.86 0.004 0.76 0.41-0.91 <0.001
0.002
9 Leg RP 0.96 0.88-0.99 <0.001 0.92 0.79-0.97 0.97 0.91-0.99 <0.001 0.90 0.73-0.97 <0.001 0.61 0.16-0.85 0.006 0.83 0.57-0.94 <0.001
<0.001
10 Leg LP 0.83 0.56-0.94 <0.001 0.88 0.70-0.96 0.89 0.70-0.96 <0.001 0.82 0.55-0.94 <0.001 0.81 0.53-0.93 <0.001 0.84 0.59-0.94 <0.001
<0.001
11 Leg RD 0.96 0.89-0.99 <0.001 0.95 0.85-0.98 0.94 0.84-0.98 <0.001 0.83 0.57-0.94 <0.001 0.73 0.36-0.90 0.001 0.80 0.50-0.93 <0.001
<0.001
12 Leg LD 0.77 0.44-0.92 <0.001 0.88 0.68-0.98 0.92 0.76-0.97 <0.001 0.88 0.68-0.96 <0.001 0.76 0.41-0.91 <0.001 0.83 0.57-0.94 <0.001
<0.001

Total score 0.95 0.86-0.95 <0.001 0.90 0.74-0.97 0.93 0.82-0.98 <0.001 0.93 0.81-0.98 <0.001 0.90 0.72-0.96 <0.001 0.93 0.80-0.98 <0.001
<0.001

∑(D+A), summation of duration and amplitude factor ; RP, right proximal ; LP, left proximal ; RD, right distal ; LD, left distal

2
Appendix S1: Patient characteristics
Age, Sex GMFCS Birth Neuroimaging findings
y:mo time
weeks
P1 15:1 M V 40 Bilateral thalamus, minimal perirolandic lesions
P2 12:6 M II 40 Normal findings
P3 7:1 M V 40 Bilateral nucleus lentiformis
P4 16:9 M V 39 Bilateral putamen, minimal minimal perirolandic
lesions
P5 17:1 M IV 40 Normal findings
P6 20:8 M II 40 Bilateral thalamus, minimal perirolandic lesions
P7 22:0 M IV 39 Normal findings
P8 15:3 M III 40 Normal findings
P9 19:0 F III 41 Bilateral putamen, globus pallidus, thalamus lesions
P10 15:0 F I 40 Unilateral putamen, globus pallidus, minimal thalamus
P11 14:5 F I 38 Bilateral putamen lesions
P12 11:7 M V 40 Bilateral nucleus lentiformis lesions
P13 7:1 M I 40 Bilateral basal ganglia, thalamus lesions
P14 11:9 M II 39 Bilateral putamen, thalamus lesions
P15 10:1 M V 35 Bilateral basal ganglia, thalamus lesions
y, years; mo, months; GMFCS, Gross Motor Function Classification system;

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