Term

Definition

Domain

Measurement property

Aspect of a measurement property

Reliability

The degree to which the measurement is free from measurement error

Reliability (extended definition)

The extent to which scores for patients who have not changed are the same for repeated measurement under several conditions; e.g. using different sets of items from the same health related-patient reported outcomes (HR-PRO) (internal consistency); over time (test-retest); by different persons on the same occasion (inter-rater; or by the same persons (i.e. raters or responders) on different occasions (intra-rater)

Internal consistency

The degree of the interrelatedness among the items

Reliability

The proportion of the total variance in the measurements which is due to ‘true’† differences between patients

Measurement

The systematic and random error of a patient’s score that is not attributed to true changes in the construct to be measured

Validity

The degree to which an HR-PRO instrument measures the construct(s) it purports to measure

Content validity

The degree to which the content of an HR-PRO instrument is an adequate reflection of the construct to be measured

Face validity

The degree to which (the items of) an HR-PRO instrument indeed looks as though they are an adequate reflection of the construct to be measured

Construct validity

The degree to which the scores of an HR-PRO instrument are consistent with hypotheses (for instance with regard to internal relationships, relationships to scores of other instruments, or differences between relevant groups) based on the assumption that the HR-PRO instrument validly measures the construct to be measured

Structural validity

The degree to which the scores of an HR-PRO instrument are an adequate reflection of the dimensionality of the construct to be measured

Hypotheses testing

Idem construct validity

Cross-cultural validity

The degree to which the performance of the items on a translated or culturally adapted HR-PRO instrument are an adequate reflection of the performance of the items of the original version of the HR-PRO instrument

Content validity

The degree to which the content of an HR-PRO instrument is an adequate reflection of the construct to be measured

Face validity

The degree to which (the items of) an HR-PRO instrument indeed looks as though they are an adequate reflection of the construct to be measured

Criterion validity

The degree to which the scores of an HR-PRO instrument are an adequate reflection of a ‘gold standard’

Responsiveness

The ability of an HR-PRO instrument to detect change over time in the construct to be measured

Responsiveness

Idem responsiveness

Interpretability*

Interpretability is the degree to which one can assign qualitative meaning – that is, clinical or commonly understood connotations – to an instrument’s quantitative scores or change in scores.

Measurement Property

Rating

Criteria

Structural validity

+

CTT: CFI or TLI or comparable measure > 0.95 OR RMSEA < 0.06 OR SRMR < 0.082

IRT/Rasch: No violation or unidimensionality: CFI or TLI or comparable measure > 0.95 OR RMSEA < 0.06 OR SRMR < 0.08

AND

no violation of local independence: residual correlations among the items after controlling for the dominant factor < 0.20 OR Q3’s < 0.37

AND

no violation of monotonicity: adequate looking graphs OR item scalability > 0.30

AND

adequate model fit IRT: X² > 0.01.

Rasch: infit and outfit means squares ≥ 0.5 and ≤ 1.5 OR Z-standardised values > -2 and < 2

?

CTT: not all information for ‘+’ reported

IRT/Rasch: model fit not reported

-

Criteria for ‘+’ not met

Internal consistency

+

At least low evidence for sufficient structural validity

AND

Cronbach’s alpha(s) ≥ 0.70 for each unidimensional scale or subscale

?

Criteria for “At least low evidence for sufficient structural validity” not met

-

At least low evidence for sufficient structural validity

AND

Cronbach’s alpha(s) < 0.70 for each unidimensional scale or subscale

Reliability

+

ICC or weighted Kappa ≥ 0.70

?

ICC or weighted Kappa not reported

-

ICC or weighted Kappa < 0.70

Measurement error

+

SDC or LoA < MIC

?

MIC not defined

-

SDC or LoA >MIC

Hypotheses testing for construct validity

+

The result is in accordance with the hypothesis

?

No hypothesis defined (by the review team)

-

The result is not in accordance with the hypothesis

Cross-cultural validity/measurement invariance

+

No important differences found between group factors (such as age, gender, language) in multiple group factor analysis OR no important DIF for group factors (McFadden’s R² <0.02)

?

No multiple group factor analysis OR DIF analysis performed

-

Important differences between group factors OR DIF was found

Criterion validity

+

Correlation with gold standard ≥ 0.70 or AUC ≥ 0.70

?

Not all information for ‘+’ reported

-

Correlation with gold standard < 0.70 or AUC < 0.70

Responsiveness

+

The result is in accordance with the hypothesis OR AUC ≥ 0.70

?

No hypothesis defined (by the review team)

-

The result is not in accordance with the hypothesis or AUC < 0.70

Study reference

Study characteristics

Participant characteristics

Measurement properties

Comments

Stewart, 2017

Systematic review, literature search up to April 2016

34 studies were included, of which 18 presented results specifically for children with CP

Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS)

A: Monbaliu, 2010

B: Marks, 2011

C: Keen, 2014

D: Motta, 2008

E: Gimeno, 2012

F: Air, 2011

G: Lumsden, 2013

H: Marks, 2013

I: Olaya, 2013

J: Romito, 2015

K: Sanger, 2007

L: Trabacca, 2011

Barry–Albright Dystonia Scale (BADS)

A: Monbaliu, 2010

D: Motta, 2008

M: Gordon, 2006

N: Kawamura, 2012

O: Monbaliu, 2012

Unified Dystonia Rating Scale (UDRS)

A: Monbaliu, 2010

K: Sanger, 2007

Movement Disorder-Childhood Rating Scale (MD-CRS)

P: Battini, 2014

MD-CRS (0-3 years)

P: Battini, 2014

Dyskinesia Impairment Scale (DIS)

O: Monbaliu, 2012

Q: Monbaliu, 2015

R: Monbaliu, 2013

Source of funding and conflicts of interest:

No funding reported. No conflicts of interest reported.

Inclusion criteria SR:

- include a clinical measure of dystonia and/or choreoathetosis according to the definition of Himmelmann (2007);

- include children with CP aged 0 to 18 years;

- report original psychometric data that could be isolated for children with CP;

- reported in English.

Exclusion criteria SR:

- reported only laboratory-based measures.

Psychometric and clinical utility data were extracted for the selected instruments reported in the included papers using the CanChild Outcome Measures Rating Form for validity, reliability, responsiveness, and clinical utility.

Measurement properties considered in the review by Stewart (2017) were:

Validity

- construct validity (structural validity, hypothesis testing, cross-cultural validity)

- content validity

- criterion validity (concurrent and predictive)

Reliability

- intra-rater

- test-retest

- inter-rater

- internal consistency

Responsiveness

- responsiveness

Reliability was evaluated as the degree to which an assessment yielded the same score when administered by the same examiner on different occasions (intrarater or test-retest), or by a different examiner on the same occasion (interrater). Reliability data presented using intra- class correlation coefficients and Cronbach’s alpha were rated using the following criteria: greater than or equal to 0.8, excellent; 0.6 to 0.79, adequate; less than or equal to 0.6, poor.

Validity was evaluated as the extent to which the tools measured what they purported to measure and included construct, content, and criterion validity.

Responsiveness, the ability of the tool to detect change over time in the construct being measured, was reported as the mean change in scale scores after an intervention and where available statistical significance of this change was noted.

Rating of level of evidence:

Stewart (2017) used the Consensus-based Standards for selection of health Measurement Instruments (COSMIN) checklist used to rate the evidence for each scale using the levels strong, moderate, limited, conflicting, or unknown.

This rating was based on:

(1) The number of studies available involving the scale;

(2) the methodological quality of each study (excellent, good, fair, poor);

(3) the strength of psychometric data (good, intermediate, poor, unknown);

(4) the finding of each study (positive or negative.

The overall quality score for each measurement property was determined by the lowest rating of any of the items as recommended by the COSMIN manual.

Author’s conclusion

Each of the six tools identified in this review provides valuable information about dystonia and/ or choreoathetosis severity in children with CP; however, only the BADS and DIS have been developed specifically for CP and only the DIS addresses both dystonia and choreoathetosis. Further studies are required to examine the validity, reliability, responsiveness, and clinical utility of each scale for use with children with CP before clear recommendations can be made about tool choice for research and clinical purposes. In the meantime, the BADS can be considered the most clinically useful tool for measuring dystonia in children with CP. For research purposes or CP register settings, the DIS presents as the most com- prehensive tool, enabling experienced users to distinguish between and measure both dystonia and choreoathetosis.

Haberfehlner (2020)

Instrument assessed:

Dyskinesia Impairment Scale

Type of study: secondary analysis of an RCT on the effects of intrathecal baclofen (ITB)

Setting and country: two university medical centres in the Netherlands

Funding and conflicts of interest: No funding was reported. The authors reported no conflicts of interest.

Inclusion criteria:

- presenting with dyskinetic CP;

- classified in Gross Motor Function Classification System levels IV and V;

- aged 4 to 25 years;

- lesions on magnetic resonance imaging;

- eligible for ITB treatment using commonly applied criteria;

- completed the DIS and BADS at baseline and 3 months after pump implantation.

Exclusion criteria:

- contraindication for general anaesthesia or baclofen;

- oral pharmacological treatment was sufficient;

- deep brain stimulation;

- ventriculoperitoneal shunt;

- other disorders interfering with treatment.

N=33

Mean age ± SD:

ITB-treated (n=17): 14y1mo ± 4y 1mo

Placebo-treated (n=16): 14y7mo ± 4y

Sex: 73% M / 27% F

Brief video sequences of all participants were made, according to the DIS video protocol. Two experienced paediatric therapists were trained to score the DIS and BADS and scored all videos. They were blinded to the measurement time points (baseline and follow-up) and group allocation and scored the videos in random order within a year’s time. The same rater assessed both time points (baseline and follow-up) and both scales (DIS and BADS) in an individual child. Scoring of the BADS was performed on a selection of videos recorded for the DIS: sitting at rest; speaking (if performed); grasping/reaching for a pen from a lying position (left and right), and rolling over left and right.

Concurrent validity

The score of the DIS and the score for the criterion standard (i.e. BADS) were considered at the same time point (at baseline and follow-up). At both time points, the correlation between the DIS dystonia subscale and the BADS was assessed.

Responsiveness

The effect sizes of the DIS were interpreted using Cohen’s criteria. Effect sizes of the DIS dystonia subscale were compared to the effect size of the BADS.

Mean follow-up time was 3 months (SD 0.2 months) in the ITB-treated group and 2.8 months (SD 0.6 months) in the placebo-treated group.

Authors’ conclusion

The DIS dystonia subscale shows similar responsiveness in comparison to the BADS in non-ambulatory children and young adults with dyskinetic CP. Concurrent validity of the DIS is adequate as reported previously. Feasibility of the DIS activity items is restricted in patients with dyskinetic CP in GMFCS levels IV and V. Reducing the activity items of the DIS should be investigated.

Vanmechelen (2020)

Instrument assessed:

Dyskinesia Impairment Scale

Type of study: subset of participants included in the study by Monbaliu (2012) who were available for a retest recording.

Setting and country: special education schools for children with motor disability in Belgium

Funding and conflicts of interest: This work was supported by a grant from the Marguerite-Marie Delacroix Foundation. The authors have stated that they had no interest that could be perceived as posing a conflict or bias.

Inclusion criteria:

-predominant dyskinetic CP diagnosed by a paediatric neurologist within a reference centre for CP and birth at term

Exclusion criteria:

-changes in muscle tone-changing medication within the last 3 months;

-orthopaedic or neurosurgical interventions within the previous year;

-spine fusion.

N=15

Mean age ± SD: 14y ± 4y, range 5-22y

Sex: 87% M / 13% F

Participants were videotaped according to the DIS video protocol, which contains three rest postures and 24 activities. During filming, male participants wore shorts and female participants wore shorts and a bra to maximize visibility of all body parts for scoring. To assess reliability over time, participants were videotaped twice, the second time within 7 days after the first recording to minimize environmental or therapy effects. Test and retest videos were filmed by the same assessor (EM). Scoring of both test and retest videos was done by the same rater (EM) within 15 days, with 3 months in between the scoring of the test and retest 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, during both 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.

Test-retest reliability (7 days)

Intraclass correlation coefficients and 95% confidence intervals, standard error of measurement and the minimal detectable difference were assessed.

Authors’ 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.

Claassen (2023)

Instrument assessed: Movement Disorder-Childhood Rating Scale

Type of study: qualitative cross-sectional interview study

Setting and country: children and adolescents were recruited from patient panels, through physician outreach, and social media. The study was conducted in the United States of America

Funding and conflicts of interest:

This study was funded by Teva Branded Pharmaceutical Products R&D, Inc., West Chester, PA, USA. The sponsor was involved in the study design, data collection and analysis, writing the report, and the decision to submit the article. R.B. thanks Current Research (RC) of the Italian Ministry of Health, IRCCS Stella Maris Foundation (FSM), entitled “Development of a Genetic and Acquired Movement Disorders Register in Children: From Creating Italian Network to Providing New Outcome Measures” and 51000 IRCCS FSM. The authors thank William J. Kelley, PhD (Cello Health Communications/ MedErgy, with funding from Teva Pharmaceuticals), for editorial assistance in the preparation of this manuscript.

Several authors reported various potential conflicts of interest.

Inclusion criteria for children and adolescents:

- children aged six to 11 years (inclusive) or adolescent aged 12 to 18 years (inclusive);

- living in the United States;

- having self-reported DCP and predominant choreiform movement or dystonia;

- have been treated for DCP;

- have a caregiver willing to provide permission for the child to participate and who is willing to participate in the study themselves as part of a child-caregiver dyad;

- able to communicate verbally;

- willing and able to participate in a 60-minute interview.

Inclusion criteria for caregivers:

- acting as a caregiver to a child or adolescent aged six to 18 years with DCP who experiences choreiform movements or dystonia;

- living in the United States;

- speaking and reading English fluently.

Caregivers were defined as anyone, paid or unpaid, who spends at least four hours per day providing care (e.g., helping child with bathing, dressing, eating, taking medication, or transportation assistance) for a child or adolescent with DCP.

N=8 children/adolescents and 12 caregivers

Children and adolescents mean age ± SD: 14y ± 3.83y

Sex: 63% M / 38% F

60-minute interviews were conducted with children and adolescents with DCP and caregivers of children with DCP.

As formative work, the study team closely reviewed all published literature specific to the MD- CRS and conducted interviews with key opinion leaders, including the developers of the MD-CRS and three neurologists who treat children with DCP, to gather insight on the instrument.

Two semi-structured interview guides were developed (version 1: ages six to 11 years, version 2: ages 12 to 18 years, version 3: caregivers).

 All interviews were conducted by the study team's qualitative researchers, who were familiar with the specific objectives and requirements of the study and trained to ensure proper interview preparation. Interviews were recorded using digital audio-recording equipment. All interview recordings were transcribed verbatim.

Content validity

Participants were asked to identify body regions affected by DCP. The interviewer probed for any regions included in the MD-CRS that were not mentioned spontaneously by participants.

Participants were asked how DCP affected their daily functioning. The interviewer probed for any functions or activities included in the MD-CRS that were not mentioned spontaneously by participants.

Caregivers were asked to reflect on the relevance and usefulness of each item and response option in Part I of the MD-CRS.

Authors’ conclusion

The information gathered from this qualitative study provides evidence in support of the content validity of the MD-CRS for children/adolescents with DCP. The children/adolescents and caregivers interviewed for this study confirmed that all body regions and activities listed in the MD-CRS were relevant to their experiences with DCP. This study suggests that the MD-CRS is fit for purpose as a clinical trial end point to assess the effect of treatment for children and adolescents with DCP.

Instrument

Psychometric property

1^st author (year of publication)

Results

For studies included in the review by Stewart (2017): Stewart’s rating of evidence using the COSMIN checklist

For the three original studies:

COSMIN Risk of bias assessment

Assessment of study findings

Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS)

Validity

Construct

-

-

-

-

Content

Monbaliu (2010)*

Expert opinion: independent authors judged the ability of BFMDRS to measure six features of the dystonic movement disorder. (1) Body regions: yes (nine regions); (2) proximal and distal limb segments: no; (3) during rest vs activity: partial; (4) duration: partial; (5) amplitude: partial; (6) influence on functional activities: yes. Overall: tool not aligned with recent dystonia definitions and considers dystonia only.

Moderate: three fair studies

n/a

Marks (2011)*

Expert opinion: scale most sensitive to change. Scales not necessarily suitable for children with CP with mixed motor type.

n/a

Keen (2014)*

Expert opinion: not appropriately tailored for children with dystonic CP with mixed motor type. Does not reflect on subtle quality of life or caretaking changes.

n/a

Criterion (concurrent)

Motta (2008)*

BADS vs BFMDRS (n=19 CP, 2y 5mo–16y 6mo): moderate correlation between total scores (r=0.59, p=0.05)

Moderate: two fair studies

n/a

Monbaliu (2010)*

BADS vs BFMDRS (n=10 CP, 5–22y): high correlation between total scores of three raters (rater 1: Pearson’s r=0.93, 95% CI 0.73–0.98; rater 2: r=0.95, 95% CI 0.79–0.99; rater 3: r=0.95, 95% CI 0.69–0.98; all p<0.001).

n/a

Criterion (predictive)

Gimeno (2012)*

BFMDRS vs COPM (n=14 CP, 3y 6mo–18y): no correlation between changes in BFMDRS score and changes in COPM after deep brain stimulation at 6mo (r=0.201, p=0.511) or 12mo (r=0.176, p=0.565).

Limited: one fair study

n/a

Reliability

Intra-rater

-

-

-

-

Test-retest

-

-

-

-

Inter-rater

Monbaliu (2010)*

Video-based assessment: (CP n=10, age 5-22 years, 3 experienced raters)
Total scores - excellent reliability for the ‘Severity factor’ (ICC_2,1=0.89) and the ‘Provoking factor x severity factor’ (ICC_2,1=0.86) and adequate reliability for the ‘Provoking factor’ (ICC_2,1=0.64)
Item scores - poor to adequate reliability for ‘Severity factor’ items (ICC_2,1=0.34-0.77); and poor to excellent reliability for ‘Provoking factor’ (ICC_2,1 = 0.32-0.93) and ‘Severity factor x Provoking factor’ items (ICC_2,1=0.45-0.86).
Measurement error - high (SEM = 9.88%)
Smallest detectable change - large (SDD = 27.39%)

Moderate: one good study

n/a

Internal consistency

Monbaliu (2010)*

Video-based assessment: (CP n=10, 5-22 years, 3 experienced raters) Excellent internal consistency (Cronbach α = 0.83-0.94)  

Moderate: one good study

n/a

Responsiveness

Air (2011)*

Intervention: Deep Brain Stimulation

Sample: CP=6 (data for CP=4 only, 6.9 to 17.8 years)
Follow-up: range 3-9m

Responsiveness:

movement scale: 9.67% mean decrease(ref*)

disability scale: 14% mean decrease

-

-

Gimeno (2012)*

Intervention: Deep Brain Stimulation

Sample: CP=6

Follow-up: 6 & 12m

Responsiveness:

movement scale: no clinical/significant change, mean decrease 5.9% (range 1.5% to 9%)

-

-

Keen (2014)*

Intervention: Deep Brain Stimulation

Sample: CP=5 (8 to 17 years)

Follow-up: mean 26.6m

Responsiveness:

movement scale: mean decrease 28.5% (p=0.1)

-

-

Lumsden (2013)*

Intervention: Deep Brain Stimulation

Sample: CP=28 (3.3 to 20 years)

Follow-up: 6 & 12 m

Responsiveness:

movement scale: median (% improvement) Pre: 99, 6m: 97 (5.3%); 12m 95.5 (7.3%)

-

-

Marks (2011)*

Intervention: Deep Brain Stimulation

Sample: CP=8 (7 to 15 years)

Follow-up: 6 m

Responsiveness:

movement scale: mean decrease 37.84%, p=0.012

disability scale: mean decrease 14.44%, p=0.02

-

-

Marks (2013)*

Intervention: Deep Brain Stimulation

Sample: CP=9 (mean 11.02 years)

Follow-up: 6, 12 & 18 m

Responsiveness:

movement scale: clinically significant 24.3% mean decrease (p=0.90)

disability scale: 3.62% mean decrease (p=0.70)

-

-

Olaya (2013)*

Intervention: Deep Brain Stimulation

Sample: CP=2 (6 and 16 years)

Follow-up: 3 m

Responsiveness:

movement scale: 0% change

-

-

Romito (2015)*

Intervention: Deep Brain Stimulation

Sample: CP=4 (15 to 18 years)

Follow-up: range 3-6.2 years

Responsiveness:

movement scale: 37.34% mean decrease

disability scale: 17.89% mean decrease

-

-

Motta (2008)*

Intervention: Intrathecal Baclofen

Sample: CP=19 (2y5m to 16y6m)

Follow-up: 12 m

Responsiveness:

movement scale: significant decrease, p<0.001

disability scale: no change

-

-

Sanger (2007)*

Intervention: Botulinum Toxin-A

Sample: CP=7 (2.1 to 15.7 years)

Follow-up: 1m post injections

Responsiveness:

movement scale: upper extremity subscore: change p=0.0069

-

-

Trabacca (2011)*

Intervention: Tetrabenazine

Sample: CP=1 (12 years)

Follow-up: 6 m

Responsiveness:

movement scale: 18% decrease in dystonia score

-

-

Barry–Albright Dystonia Scale (BADS)

Validity

Construct

Gordon (2006)*

Clinical studies

Spasticity: Ashworth scale (n=13 CP). Higher dystonia not correlated with greater spasticity (r=0.36, p=0.22).

Moderate: three fair studies

n/a

Gordon (2006)*

Laboratory studies
Motor control: kinematic analysis of reaching (n=13 CP). Higher BADS score correlated with longer reach paths (r=0.75, p=0.005).

n/a

Kawamura (2012)*

Motor control: Kinematic Dystonia Measure (n=8 CP). Poorer hand- tapping correlated with higher total BADS score (r=0.79, p=0.003) and affected arm BADS score (r=0.76, p=0.007). Slower eye-blinking correlated with adjusted affected arm BADS scores (r=0.72, p=0.02) but not total BADS score (r=0.41, p=0.24).

n/a

Content

Monbaliu (2010)*

Expert opinion: ability to measure (1) body regions: yes (eight regions); (2) proximal and distal limb segments: no; (3) during rest vs activity: no; (4) duration: yes; (5) amplitude: yes; (6) influence on functional activities: yes. Overall: tool not aligned with recent dystonia definitions and considers dystonia only.

Limited: one fair study

n/a

Criterion (concurrent)

Motta (2008)*

BFMDRS vs BADS (n=19 CP, 2y 5mo–16y 6mo): moderate correlation between total scores (r=0.59, p=0.05).

Moderate: four fair-good studies

n/a

Monbaliu (2010)*

BFMDRS vs BADS (n=10 CP, 5–22y): high correlation between total scores of three raters (rater 1: Pearson’s r=0.93, 95% CI 0.73–0.98; rater 2: r=0.95, 95% CI 0.79–0.99; rater 3: r=0.95, 95% CI 0.69–0.98; all p<0.001).

n/a

Monbaliu (2012)*

DIS vs BADS (n=25 CP, 5–22y): good correlation between scales (r=0.84; 95% CI 0.66–0.92; p<0.001).

n/a

Monbaliu (2010)*

UDRS vs BADS (n=10 CP, 5–22y): moderate–high correlation between total scores of three raters (rater 1: r=0.89, 95% CI 0.60–0.97, p<0.001; rater 2: r=0.84, 95% CI 0.6–0.96, p=0.002; rater 3: r=0.86, 95% CI 0.51–0.97, p<0.001).

n/a

Criterion (predictive)

Battini (2008)*

BADS vs ‘global rating of change’ (before and after trial) (n=10 CP, 3–15y): assessed by doctor, physical therapist, and family after intrathecal baclofen therapy trial; agreement same (n=1), worse (n=1), better (n=8).

Limited: one fair study

n/a

Reliability

Intra-rater

-

No data specifically for children with CP

-

-

Test-retest

-

No data specifically for children with CP

-

-

Inter-rater

Monbaliu (2010)*

Video-based assessment: (CP n=10, age 5-22 years, 3 experienced raters)
Total score - excellent reliability (ICC_2,1=0.87).
Item scores - adequate reliability for limbs and trunk (ICC_2,1=0.61-0.76); but poor for eyes, mouth & neck (ICC_2,1=0.39-0.57).
Measurement error - high (SEM = 6.36%)
Smallest detectable change - large (SDD = 17.72%)

Moderate: one good study

n/a

Internal consistency

Monbaliu (2010)*

Video-based assessment: (CP n=10, 5-22 years, 3 experienced raters) Excellent internal consistency (Cronbach α = 0.87-0.91)

Moderate: one good study

n/a

Responsiveness

Air (2011)*

Intervention: Deep Brain Stimulation

Sample: CP=1 (8 years)

Follow-up: 9 m

Responsiveness: mean decrease 21.7% (no p-value)

-

-

Bhanpuri (2014)*

Intervention: Deep Brain Stimulation

Sample: CP=3 (8 to 17 years)

Follow-up: varied, >5 m

Responsiveness: no mean change (no p-value)

-

-

Marks (2011)*

Intervention: Deep Brain Stimulation

Sample: CP=8 (7 to 15 years)

Follow-up: 6 m

Responsiveness: mean decrease 19.48%, p=0.024

-

-

Albright (2009)*

Intervention: Intra-ventricular Baclofen

Sample: CP=4 (2 to 10 years)

Follow-up: range 3-21 months

Responsiveness: mean decrease 54.87% (no p-value)

-

-

Barry (1999)*

Intervention: Intrathecal Baclofen

Sample: CP=10 (3 to 15 years)

Follow-up: range 3-9 days

Responsiveness: mean decrease 47.3% (no p-value)

-

-

Bollo (2012)*

Intervention: Intrathecal Baclofen

Sample: CP=2 (3.7 and 17 years)

Follow-up: 6 m

Responsiveness: mean decrease 62.15% (no p-value)

-

-

Motta (2008)*

Intervention: Intrathecal Baclofen

Sample: CP=19 (2y5m to 16y6m)

Follow-up: 12 m

Responsiveness: significant decrease, p<0.001; mean baseline=23.84, mean 12 months post=17.79

-

-

Motta (2011)*

Intervention: Intrathecal Baclofen

Sample: CP=7 (mean 15y11m, SD 10y8m)

Follow-up: 12 m

Responsiveness: median decrease 21 (IQR 10) to 15 (IQR 4) (p=0.018)

-

-

Ward (2009)*

Intervention: Intrathecal Baclofen

Sample: CP=4 (3-16 years)

Follow-up: 6 m

Responsiveness: mean decrease 44.82% (no p-value)

-

-

Dachy (2004)*

Intervention: Baclofen test bolus

Sample: CP=5 (2 to 16 years)

Follow-up: immediate

Responsiveness: mean baseline=16.6, mean post test dose=14.8

-

-

Rice (2009)*

Intervention: Artane

Sample: CP=16 (2 to 17 years)

Follow-up: 12 and 28 weeks

Responsiveness: mean baseline=18.4, mean end of drug phase=18.3, mean end of placebo phase=16.9

-

-

Unified Dystonia Rating Scale (UDRS)

Validity

Construct

-

-

-

-

Content

Monbaliu (2010)*

Expert opinion: ability to measure (1) body regions: yes (14 regions); (2) proximal and distal limb segments: yes; (3) during rest vs activity: no; (4) duration: partial; (5) amplitude: yes; (6) influence on functional activities. Overall: tool not aligned with recent dystonia definitions and considers dystonia only.

Moderate: one good study

n/a

Criterion (concurrent)

Monbaliu (2010)*

BADS vs UDRS (n=10 CP, 5–22y): moderate–high correlation between total scores of three raters (rater 1: r=0.89, 95% CI 0.60–0.97, p<0.001; rater 2: r=0.84, 95% CI 0.6–0.96, p=0.002; rater 3: r=0.86, 95% CI 0.51–0.97, p<0.001)

BFMDRS vs UDRS (n=10 CP, 5–22y): high correlation between total scores (rater 1: Pearson’s r=0.87, 95% CI 0.53– 0.97, p<0.001; rater 2: r=0.88, 95% CI 0.56–0.97, p=0.002; rater 3: r=0.93, 95% CI 0.73–0.98, p<0.001).

Moderate: one good study, two comparisons

n/a

Reliability

Intra-rater

-

-

-

-

Test-retest

-

-

-

-

Inter-rater

Monbaliu (2010)*

Video-based assessment: (CP n=10, age 5-22 years, 3 experienced raters)
Total scores - adequate reliability for all totals (‘Duration Factor’ ICC_2,1=0.74; ‘Motor Severity’ ICC_2,1=0.79; and ‘Duration Factor x Motor Severity Factor’ ICC_2,1=0.79).
Item scores - poor to adequate reliability for ‘Duration Factor’ items (ICC_2,1=0.17-0.71); and poor to excellent reliability for ‘Motor Severity Factor’ items (ICC_2,1=0.26-0.83) and Duration Factor x Motor Severity Factor’ items (ICC_2,1=0.37-0.81).
Measurement error - high (SEM = 8.89%)
Smallest detectable difference - large (SDD = 24.63%)

Moderate: one good study

n/a

Internal consistency

Monbaliu (2010)*

Video-based assessment: (CP n=10, 5-22 years, 3 experienced raters) Excellent internal consistency (Cronbach α = 0.90-0.95)

Moderate: one good study

n/a

Responsiveness

Sanger (2007)*

Study: Botulinum Toxin-A

Sample: CP=7 (2.1 to 15.7 years)

Follow-up: 1 month post injections

Responsiveness: proximal upper extremity change p=0.12, distal change p=0.00088

-

-

Movement Disorder-Childhood Rating Scale (MD-CRS)

Validity

Construct

-

-

-

-

Content

Claassen (2023)

Interviews with children/adolescents and caregivers: 

Participants confirmed that the body regions and activities listed in the MD-CRS were affected by DCP and that involuntary movements interfered with all motor, oral/verbal, self-care, and video protocol activities. Caregivers endorsed the response options for 12 of 15 items in MD-CRS Part I and suggested clarifications for others.

n/a

Methodological quality:

adequate

sufficient content validity

Criterion (concurrent or predictive)

-

-

-

-

Reliability

Intra-rater

-

-

-

-

Test-retest

-

No data specifically for children with CP

-

-

Inter-rater

-

No data specifically for children with CP

-

-

Internal consistency

-

No data specifically for children with CP

-

-

Responsiveness

Battini (2014)*

Study: Trihexyphenidyl trial

Sample: CP=27 (4 to 18.3 years)

Follow-up: 6 and 12 months

Responsiveness:

ANOVA main effect of time for all 3 Indices significant, p<0.0001

Bonferroni post-hoc test on change scores significant between all time points for all indices (expect Index II at 12m vs 6m)

Regression analysis: not significant (p>0.05) for changes at 12m & age for all Indices.

-

-

Movement Disorder-Childhood Rating Scale 0-3 years (MD-CRS 0-3)

Validity

Construct

-

-

-

-

Content

-

-

-

-

Criterion (concurrent or predictive)

-

-

-

-

Reliability

Intra-rater

-

-

-

-

Test-retest

-

No data specifically for children with CP

-

-

Inter-rater

-

No data specifically for children with CP

-

-

Internal consistency

-

No data specifically for children with CP

-

-

Responsiveness

Battini (2014)*

Study: Trihexyphenidyl trial

Sample: CP=20 (0.7 to 3.3 years)

Follow-up: 6 and 12 months

Responsiveness:

ANOVA main effect of time for all 3 Indices significant, p<0.0001

Bonferroni post-hoc test on change scores significant between all time points for all indices (expect Index II at 12m vs 6m)

Regression analysis: not significant (p>0.05) for changes ta 12m & age for all Indices.

-

-

Dyskinesia Impairment Scale (DIS)

Validity

Construct

-

-

-

-

Content

Monbaliu (2012)*

Expert opinion sought on content and available scales

Limited: one fair study

n/a

Criterion (concurrent)

Monbaliu (2012)*

DIS vs BADS (n=25 CP, 5–22y): good correlation between scales (r=0.84; 95% CI 0.66–0.92; p<0.001.

Limited: one fair study

n/a

Haberfehlner (2020)

The Pearson’s correlation coefficients between the DIS dystonia subscale and BADS at baseline and follow-up were 0.78 (95% CI 0.59–0.88; p<0.001) and 0.79 (95% CI 0.60–0.89; p<0.001) respectively.

n/a

Methodological quality: very good

Sufficient validity (correlations ≥ 0.70)

Criterion (predictive)

Monbaliu (2015)*

DIS vs GMFCS, MACS, and CFCS (n=55 CP, 6–22y). Higher GMFCS showed good correlation with higher total dystonia score (Spearman’s q=0.70, 95% CI 0.53–0.81, p<0.001) and dystonia in the leg region (q=0.65, 95% CI 0.47– 0.78, p<0.01). Higher MACS showed good correlations with total dystonia (q=0.68, 95% CI 0.47–0.81, p<0.001) and dystonia in the arm region (q=0.74, 95% CI 0.59–0.84, p<0.001). Higher CFCS showed fair correlation with total DIS (q=0.36, 95% CI 0.11–0.57, p=0.002) and dystonia in the mouth region (q=0.36, 95% CI 0.11–0.57, p=0.002). No significant correlations were found between choreoathetosis and GMFCS (total p=0.18, leg region p=0.86); or MACS (total p=0.09, arm region p=0.08); or CFCS (total p=0.06; mouth region p=0.30).

Limited: one fair study

n/a

Reliability

Intra-rater

-

-

-

-

Test-retest

Vanmechelen (2020)

Intraclass correlation coefficients of the DIS, the dystonia subscale of the DIS, 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%.

n/a

Methodological quality:

doubtful

sufficient test-retest reliability (ICC ≥ 0.70)

Inter-rater

Monbaliu (2012)*

Video-based assessment: (CP n=25, age 5-22 years, 2 experienced raters)
Total scores - excellent reliability overall (ICC=0.96) and for the subscales of ‘Dystonia’ (ICC=0.91) and ‘Choreoathetosis’ (ICC=0.98).
Regional items - poor to excellent reliability for ‘Dystonia’ (ICC=0.46-1.00) and ‘Choreoathetosis’ (ICC=0.48-0.96) items.
Measurement error - low for the Total DIS (SEM=3%) and both the ‘Dystonia’ (SEM=5%) and ‘Choreoathetosis’ (SEM=3%) subscales.
Minimal detectable difference - moderate for the Total DIS (MDD=9%) and ‘Choreoathetosis’ subscale (MDD=7%); and large for the ‘Dystonia’ subscale (MDD=15%)

Moderate: two good studies

n/a

Monbaliu (2013)*

Video-based assessment: (CP n=25, age 5-22 years, 5 inexperienced raters - 3 senior and 2 junior PTs)
Junior PTs
Total scores - excellent reliability for the Total DIS (ICC=0.88) and both subscales (Dystonia: ICC=0.82, Choreoathetosis: ICC=0.88).
Measurement error – moderate for the Total DIS (SEM=5.5%) and both subscales (Dystonia SEM=6.9%; Choreoathetosis SEM=6.4%).
Minimal detectable difference - large for the Total DIS (MDD=15.3%) and both subscales (Dystonia MDD=18.4%; Choreoathetosis MDD=17.6%).
Senior PTs
Total scores - excellent reliability for the Total DIS (ICC=0.88) and the ‘Choreoathetosis’ subscale (ICC=0.91), but only adequate reliability for the ‘Dystonia’ subscale (ICC=0.71).
Measurement error - low for the Total DIS (SEM=4.4%) and ‘Choreoathetosis’ subscale (SEM=5%) and moderate for the ‘Dystonia’ subscale (SEM=7.7%);
Minimal detectable difference - large for the Total DIS (SEM=12.1%) and both subscales (Dystonia MDD=21.3%; Choreoathetosis SEM=13.9%)

n/a

Internal consistency

Monbaliu (2012)*

Video-based assessment: (CP n=25, age 5-22 years, 2 experienced raters).
Internal consistency was excellent for the ‘Dystonia’ subscale (Cronbach α=0.90-0.93). ; and excellent for the ‘Choreoathetosis’ subscale (α=0.89 0.94)

Moderate: one good study

n/a

Monbaliu (2013)*

Video-based assessment: (CP n=25, age 5-22 years, 5 inexperienced raters). Excellent internal consistency for items across the total DIS and within Dystonia and Choreoathetosis subsections for Junior PTs (α=0.87-0.95) and Senior PTs (α=0.76 -0.93)

n/a

Responsiveness

-

Haberfehlner (2020)

Dystonia subscale

In the ITB-treated group, the effect size for the DIS dystonia subscale was 0.22 (small favourable effect), while in the placebo-treated group the effect size was +0.34 (small unfavourable effect).

The effect size of the BADS in the ITB-treated group was comparable to the effect size for the DIS dystonia subscale. However, for the placebo-treated group the effect size on the DIS dystonia subscale showed a small unfavourable effect, which was not measured on the BADS. The correlation of change scores between the DIS dystonia subscale and BADS was 0.64 (95% confidence interval (CI) 0.37–0.80; p<0.001)

Choreoathetosis subscale

The effect size in the ITB-treated group for the total DIS choreoathetosis subscale was 0.25 (small favourable effect). In the placebo-treated group, the effect size was +0.10 (no effect)

Both subscales showed no floor or ceiling effects.

n/a

Methodological quality: doubtful

Comparison with BADS:

insufficient responsiveness

Comparison between ITB group and placebo group:

sufficient responsiveness

Before and after the intervention:

insufficient responsiveness

Criterion validity (concurrent)

Author: Haberfehlner (2020)

Instrument: Dyskinesia Impairment Scale

very good

adequate

doubtful

inadequate

NA

Statistical methods

For continuous outcomes: were correlations, or the area under the receiver operating curve calculated?

Correlations or AUC calculated

Correlations or AUC NOT calculated

Not applicable

For dichotomous outcomes: were sensitivity and specificity determined?

Sensitivity and specificity calculated

Sensitivity and specificity NOT calculated

Not applicable

Other

Were there any other important flaws in the design or statistical methods of the study?

No other important methodological flaws

Other minor methodological flaws

Other important methodological flaws

Responsiveness - construct approach (i.e. hypothesis testing: comparison with other outcome measurement instruments [BADS])

Author: Haberfehlner (2020)

Instrument: Dyskinesia Impairment Scale

very good

adequate

doubtful

inadequate

NA

Design requirements

Is it clear what the comparator instrument(s) measure(s)?

Constructs measured by the comparator instrument(s) is clear

Constructs measured by the comparator instrument(s) is not clear

Were the measurement properties of the comparator instrument(s) sufficient?

Sufficient measurement properties of the comparator instrument(s) in a population similar to the study population

Sufficient measurement properties of the comparator instrument(s) but not sure if these apply to the study population

Some information on measurement properties of the comparator instrument(s) in any study population

NO information on the measurement properties of the comparator instrument(s) OR evidence of poor quality of comparator instrument(s)

Statistical methods

Was the statistical method appropriate for the hypotheses to be tested?

Statistical method was appropriate

Assumable that statistical method was appropriate

Statistical method applied NOT optimal

Statistical method applied NOT appropriate

Other

Were there any other important flaws in the design or statistical methods of the study?

No other important methodological flaws

Other minor methodological flaws

Other important methodological flaws

Responsiveness - construct approach (i.e. hypotheses testing: comparison between subgroups [ITB group and placebo group])

Author: Haberfehlner (2020)

Instrument: Dyskinesia Impairment Scale

very good

adequate

doubtful

inadequate

NA

Design requirements

Was an adequate description provided of important characteristics of the subgroups?

Adequate description of the important characteristics of the subgroups

Adequate description of most of the important characteristics of the subgroups

Poor or no description of the important characteristics of the subgroups

Statistical methods

Was the statistical method appropriate for the hypotheses to be tested?

Statistical method was appropriate

Assumable that statistical method was appropriate

Statistical method applied NOT optimal

Statistical method applied NOT appropriate

Other

Were there any other important flaws in the design or statistical methods of the study?

No other important methodological flaws

Other minor methodological flaws

Other important methodological flaws

Responsiveness - construct approach [i.e. hypotheses testing: before and after the intervention]

Author: Haberfehlner (2020)

Instrument: Dyskinesia Impairment Scale

very good

adequate

doubtful

inadequate

NA

Design requirements

Was an adequate description provided of the intervention given?

Adequate description of the intervention

Poor description of the intervention

NO description of the intervention

Statistical methods

Was the statistical method appropriate for the hypotheses to be tested?

Statistical method was appropriate

Assumable that statistical method was appropriate

Statistical method applied NOT optimal

Statistical method applied NOT appropriate

Other

Were there any other important flaws in the design or statistical methods of the study?

No other important methodological flaws

Other minor methodological flaws

Other important methodological flaws

Reliability

Author: Vanmechelen (2020)

Instrument: Dyskinesia Impairment Scale

very good

adequate

doubtful

inadequate

NA

Design requirements

Were patients stable in the interim period on the construct to be measured?

Evidence provided that patients were stable

Assumable that patients were stable

Unclear if patients were stable

Patients were NOT stable

Was the time interval appropriate?

Time interval appropriate

Doubtful whether time interval was appropriate or time interval was not stated

Time interval NOT appropriate

Were the test conditions similar for the measurements? E.g. type of administration, environment, instructions

Test conditions were similar (evidence provided)

Assumable that test conditions were similar

Unclear if test conditions were similar

Test conditions were NOT similar

Statistical methods

For continuous scores: was an intraclass correlation coefficient (ICC) calculated?

ICC calculated and model or formula of the ICC is described

ICC calculated but model or formula of the ICC not described or not optimal. Pearson or Spearman correlation coefficient calculated with evidence provided that no systematic change has occurred.

Pearson or Spearman correlation coefficient calculated WITHOUT evidence provided that no systematic change has occurred or WITH evidence that systematic change has occurred

No ICC or Pearson or Spearman correlations calculated

Not applicable

For dichotomous/nominal/ordinal scores: was kappa calculated?

Kappa calculated

No kappa calculated

Not applicable

For ordinal scores: was a weighted kappa calculated?

Weighted kappa calculated

Unweighted Kappa calculated or not described

Not applicable

For ordinal scores: was the weighting scheme described? E.g. linear, quadratic

Weighting scheme described

Weighting scheme NOT described

Not applicable

Other

Were there any other important flaws in the design or statistical methods of the study?

No other important methodological flaws

Other minor methodological flaws

Other important methodological flaws

Content validity

Author: Claassen (2023)

Instrument: Movement Disorder-Childhood Rating Scale

very good

adequate

doubtful

inadequate

NA

Asking patients about relevance

Design requirements

Was an appropriate method used to ask patients whether each item is relevant for their experience with the condition?

Widely recognized or well justified method used

Only quantitative (survey) method(s) used or assumable that the method was appropriate but not clearly described

Not clear if patients were asked whether each item is relevant or doubtful whether the method was appropriate

Methods used not appropriate or patients not asked about the relevance of all items

Was each item tested in an appropriate number of patients?

    For qualitative studies

    For quantitative (survey)

    studies

≥ 7

≥ 50

4-6

≥30

<4 or not clear

<30 or not clear

Were skilled group moderators/interviewers used?

Skilled group moderators/interviewers used

Group moderators/interviewers had limited experience or were trained specifically for the study

Not clear if group moderators/interviewers were trained or group moderators/interviewers not trained and no experience

Not applicable

Were the group meetings or interviews based on an appropriate topic or interview guide?

Appropriate topic or interview guide

Assumable that the topic or interview guide was appropriate, but not clearly described

Not clear if a topic guide was used or doubtful if topic or interview guide was appropriate or no guide

Not applicable

Were the group meetings or interviews recorded and transcribed verbatim?

All group meetings or interviews were recorded and transcribed verbatim

Assumable that all group meetings or interviews were recorded and transcribed verbatim, but not clearly described

Not clear if all group meetings or interviews were recorded and transcribed verbatim or recordings not transcribed verbatim or only notes were made during the group meetings/interviews

No recordings and no notes

Not applicable

Analyses

Was an appropriate approach used to analyse the data?

A widely recognized or well justified approach was used

Assumable that the approach was appropriate, but not clearly described

Not clear what approach was used or doubtful whether the approach was appropriate

Approach not appropriate

Were at least two researchers involved in the analysis?

At least two researchers involved in the analysis

Assumable that at least two researchers were involved in the analysis, but not clearly described

Not clear if two researchers were included in the analysis or only one researcher involved in the analysis

Quality level

Definition

High

We are very confident that the true measurement property lies close to that of the estimate* of the measurement property

Moderate

We are moderately confident in the measurement property estimate: the true measurement property is likely to be close to the estimate of the measurement property, but there is a possibility that it is substantially different

Low

Our confidence in the measurement property estimate is limited: the true measurement property may be substantially different from the estimate of the measurement property

Very low

We have very little confidence in the measurement property estimate: the true measurement property is likely to be substantially different from the estimate of the measurement property

Instrument and reference to original paper

Intended population (age range)

Body parts

Scale

Administration/clinical utility

Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS)

Burke (1985)

Primary dystonia (adults, age not specified)

n=9: eyes, mouth, speech/ swallow, neck, trunk, four limbs

Score: each body part; scores halved for mouth, eyes, neck

Movement scale (120 points)

For each body part: severity rating, provoking factor(s), severity x provoking factor

Disability scale (30 points)

Rates the patient’s view of the impact of dystonia on activities of daily including: speech, handwriting, feeding, eating/swallowing, hygiene, dressing, and walking.

Manual: nil

Administration instructions: adequatea

Administration time: NS (video recording time for items 4min 15s)

Scoring mode: face-to-face or video (protocol in journal)

Scoring criteria: in journal

Scoring time: NS

Training requirements: NS

Interpretability: higher score means greater severity

Barry–Albright Dystonia Scale (BADS)

Barry (1999)

Secondary dystonia
CP and ABI
(children and
adults)

n=8: eyes, mouth, neck, trunk, four limbs

Score: each body part

Total BADS (32 points)

Manual: nil

Administration instructions: adequate

Administration time: NS

Scoring mode: face-to-face or video (protocol in journal)

Scoring criteria: in journal

Scoring time: NS

Training requirements: training improves reliability, details not provided

Interpretability: higher score means greater severity

Unified Dystonia Rating Scale (UDRS)

Comella (2003)

Primary dystonia
(adults 18–65y)

n=14: eyes/
upper face,
lower face,
jaw/tongue, larynx,
neck, trunk,
four limbs,
proximal, and distal

Score: each body part

Total UDRS (112 points)

Duration scale (56 points)

Severity scale (56 points)

Manual: nil

Administration instructions: adequate

Administration time: NS (video

recording time for items about 5min)

Scoring mode: recommended video (protocol in journal)

Scoring criteria: in journal

Scoring time: NS

Training requirements: NS; experience improves reliability

Interpretability: higher score means greater severity

Movement Disorder-Childhood Rating Scale (MD-CRS)

Battini (2008)

CP and other
disorders Hypokinetis–rigid syndrome; chorea/ ballism; dystonia/ athetosis; myoclonus; tic; tremor (children 4– 18y)

n=7: eye and
periorbital
region, face, tongue and perioral region, neck, trunk, four limbs

Score: global index calculated (0–1): 0–0.2, healthy;

0.2–0.4, mildly affected;

0.4–0.6, moderately affected;

0.6–0.8, severely affected;

0.8–1, profoundly affected

Manual: nil

Administration instructions: adequate

Administration time: NS (video recording time for items about 20min)

Scoring mode: recommended video (protocol in journal)

Scoring criteria: in journal, statistical analysis required

Scoring time: NS

Training requirements: NS

Interpretability: higher score means greater severity

Movement Disorder-Childhood Rating Scale 0-3 years (MD-CRS 0-3)

Battini (2009)

CP and other
disorders Hypokinetis–rigid
syndrome; chorea/
ballism; dystonia/
athetosis; myoclonus; tic;
tremor (children 0–
3y)

n=7: eye and periorbital
region, face, tongue and perioral region,
neck, trunk,
four limbs

Score: global index calculated (0–1): 0–0.2, healthy;

0.2–0.4, mildly affected;

0.4–0.6, moderately affected;

0.6–0.8, severely affected;

0.8–1, profoundly affected

Manual: nil

Administration instructions: adequate Administration time: NS

Scoring mode: recommended video (protocol in journal)

Scoring criteria: in journal, statistical analysis required

Scoring time: NS

Training requirements: NS

Interpretability: higher score means greater severity

Dyskinesia Impairment Scale (DIS)

Monbaliu (2012)

Dyskinetic CP
Dystonia and choreoathetosis
subscales (children
and adults 5–22y)

n=12: eyes,
mouth,
neck, trunk,
four limbs, proximal,
and distal

Score: each body part

Total DIS (288 points)

On action (192 points)

At rest (96 points)

Dystonia Choreoathetosis

Manual: nil

Administration instructions: adequate Administration time: video recording

time maximum 30min; ROM also measurement required

Scoring mode: recommended video (protocol in journal)

Scoring criteria: in journal

Scoring time: 30–45min per subscale Training requirements: clinical

experience with dyskinesia

Interpretability: higher score means greater severity

Validiteit

Betrouwbaarheid

Responsiviteit

Construct-validiteit

Inhouds-validiteit

Criterium-validiteit (concurrent)

Criterium-validiteit (predictief)

Intra-rater

Test-hertest

Inter-rater

Interne consistentie

Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS)

Geen informatie

Redelijk bewijs (+)

Bewijs alleen gebaseerd op expert opinion, meerdere kritische kanttekeningen (?)

Redelijk bewijs

Beperkt bewijs

Geen informatie

Geen informatie

Redelijk bewijs

Redelijk bewijs (+)

Goede tot uitstekende interne consistentie (+)

Beperkt bewijs

Barry–Albright Dystonia Scale (BADS)

Redelijk bewijs

Beperkt bewijs (+)

Bewijs alleen gebaseerd op expert opinion, instrument sluit niet goed aan bij definitie dystonie, alleen gericht op dystonie (?)

Redelijk bewijs

Beperkt bewijs

Geen informatie

Geen informatie

Redelijk bewijs

Redelijk bewijs (+)

Goede tot uitstekende interne consistentie (+)

Beperkt bewijs

Unified Dystonia Rating Scale (UDRS)

Geen informatie

Redelijk bewijs (+)

Bewijs alleen gebaseerd op expert opinion, instrument sluit niet goed aan bij definitie dystonie, alleen gericht op dystonie (?)

Redelijk bewijs

Geen informatie

Geen informatie

Geen informatie

Redelijk bewijs

Redelijk bewijs (+)

Uitstekende interne consistentie (+)

Beperkt bewijs

Movement Disorder-Childhood Rating Scale (MD-CRS)

Geen informatie

Redelijk bewijs (+)

Inhoudsvaliditeit lijkt voldoende op basis van interviews met kinderen/adolescenten en hun verzorgers (+)

Geen informatie

Geen informatie

Geen informatie

Geen informatie

Geen informatie

Geen informatie (-)

Beperkt bewijs

Movement Disorder-Childhood Rating Scale 0-3 years (MD-CRS 0-3)

Geen informatie

Geen informatie (-)

Geen informatie

Geen informatie

Geen informatie

Geen informatie

Geen informatie

Geen informatie (-)

Beperkt bewijs

Dyskinesia Impairment Scale (DIS)

Geen informatie

Beperkt bewijs (+)

Bij ontwikkeling van de schaal zijn negen experts betrokken geweest om de inhoudsvaliditeit te waarborgen (?)

Redelijk bewijs

Beperkt bewijs

Geen informatie

Beperkt bewijs

Redelijk bewijs

Redelijk bewijs (+)

Adequate tot goede of uitstekende interne consistentie (+)

Beperkt bewijs

Reference

Reason for exclusion

Pavone L, Burton J, Gaebler-Spira D. Dystonia in childhood: clinical and objective measures and functional implications. J Child Neurol. 2013 Mar;28(3):340-50. doi: 10.1177/0883073812444312. Epub 2012 Jun 29. PMID: 22752485.

Wrong publication type: narrative review (older than the included systematic review by Stewart (2017))

Vanmechelen I, Danielsson A, Lidbeck C, Tedroff K, Monbaliu E, Krumlinde-Sundholm L. The Dyskinesia Impairment Scale, Second Edition: Development, construct validity, and reliability. Dev Med Child Neurol. 2023 May;65(5):683-690. doi: 10.1111/dmcn.15444. Epub 2022 Oct 31. PMID: 36310446.

Wrong index test: second version of the Dyskinesia Impairment scale, more research needed before this scale can be used in clinical practice

Kuiper MJ, Meiners LC, Chandler ES, Brandsma R, Bos AF, Horst HT, Sival DA; Neonatal neurologic Assessment Group. Dyskinesia Impairment Scale scores in Dutch pre-school children after neonatal therapeutic hypothermia. Eur J Paediatr Neurol. 2020 Sep;28:70-76. doi: 10.1016/j.ejpn.2020.07.013. Epub 2020 Aug 5. PMID: 32950367.

Wrong population: children at risk of developing dyskinetic CP

Rice J, Skuza P, Baker F, Russo R, Fehlings D. Identification and measurement of dystonia in cerebral palsy. Dev Med Child Neurol. 2017 Dec;59(12):1249-1255. doi: 10.1111/dmcn.13502. Epub 2017 Aug 8. PMID: 28786476.

Wrong population: children with CP but not all children have dystonia

Danielsson A, Vanmechelen I, Lidbeck C, Krumlinde-Sundholm L, Ortibus E, Monbaliu E, Tedroff K. Reliability and Validity of the Dyskinesia Impairment Scale in Children and Young Adults with Inherited or Idiopathic Dystonia. J Clin Med. 2020 Aug 11;9(8):2597. doi: 10.3390/jcm9082597. PMID: 32796550; PMCID: PMC7463647.

Wrong population: children and youth with inherited or idiopathic dystonia

Stewart K, Lewis J, Wallen M, Bear N, Harvey A. The Dyskinetic Cerebral Palsy Functional Impact Scale: development and validation of a new tool. Dev Med Child Neurol. 2021 Dec;63(12):1469-1475. doi: 10.1111/dmcn.14960. Epub 2021 Jun 19. PMID: 34145577.

Wrong index test: Dyskinetic Cerebral Palsy Functional Impact Scale

Battini R, Guzzetta A, Sgandurra G, Di Pietro R, Petacchi E, Mercuri E, Giannini MT, Leuzzi V, Cioni G. Scale for evaluation of movement disorders in the first three years of life. Pediatr Neurol. 2009 Apr;40(4):258-64. doi: 10.1016/j.pediatrneurol.2008.11.003. PMID: 19302937.

Included in the systematic review by Stewart (2017)

Battini R, Casarano M, Sgandurra G, Olivieri I, Di Pietro R, Romeo DM, Mercuri E, Cioni G. Responsiveness of the MD-childhood rating scale in dyskinetic cerebral palsy patients undergoing anticholinergic treatment. Eur J Paediatr Neurol. 2014 Nov;18(6):698-703. doi: 10.1016/j.ejpn.2014.06.004. Epub 2014 Jun 19. PMID: 25022341.

Included in the systematic review by Stewart (2017)

Battini R, Olivieri I, Di Pietro R, Casarano M, Sgandurra G, Romeo DM, Cioni G. Movement Disorder-Childhood Rating Scale: A Sensitive Tool to Evaluate Movement Disorders. Pediatr Neurol. 2015 Jul;53(1):73-7. doi: 10.1016/j.pediatrneurol.2015.02.014. Epub 2015 Mar 27. PMID: 26092416.

Included in the systematic review by Stewart (2017)

Elze MC, Gimeno H, Tustin K, Baker L, Lumsden DE, Hutton JL, Lin JP. Burke-Fahn-Marsden dystonia severity, Gross Motor, Manual Ability, and Communication Function Classification scales in childhood hyperkinetic movement disorders including cerebral palsy: a 'Rosetta Stone' study. Dev Med Child Neurol. 2016 Feb;58(2):145-53. doi: 10.1111/dmcn.12965. Epub 2015 Nov 30. PMID: 26616635.

Included in the systematic review by Stewart (2017)

Kawamura A, Klejman S, Fehlings D. Reliability and validity of the kinematic dystonia measure for children with upper extremity dystonia. J Child Neurol. 2012 Jul;27(7):907-13. doi: 10.1177/0883073812443086. Epub 2012 Apr 25. PMID: 22535705.

Included in the systematic review by Stewart (2017)

Monbaliu E, Ortibus E, Roelens F, Desloovere K, Deklerck J, Prinzie P, de Cock P, Feys H. Rating scales for dystonia in cerebral palsy: reliability and validity. Dev Med Child Neurol. 2010 Jun;52(6):570-5. doi: 10.1111/j.1469-8749.2009.03581.x. Epub 2010 Jan 28. PMID: 20132143.

Included in the systematic review by Stewart (2017)

Monbaliu E, Ortibus E, De Cat J, Dan B, Heyrman L, Prinzie P, De Cock P, Feys H. The Dyskinesia Impairment Scale: a new instrument to measure dystonia and choreoathetosis in dyskinetic cerebral palsy. Dev Med Child Neurol. 2012 Mar;54(3):278-83. doi: 10.1111/j.1469-8749.2011.04209.x. PMID: 22428172.

Included in the systematic review by Stewart (2017)

Monbaliu E, Ortibus E, Prinzie P, Dan B, De Cat J, De Cock P, Feys H. Can the Dyskinesia Impairment Scale be used by inexperienced raters? A reliability study. Eur J Paediatr Neurol. 2013 May;17(3):238-47. doi: 10.1016/j.ejpn.2012.10.004. Epub 2012 Nov 22. PMID: 23177615.

Included in the systematic review by Stewart (2017)