Objective: In healthy children (4-16 years of age), we aimed to associate DIS scores with age, and compare age-related influences on dyskinesia (DIS), dystonia (BFMDRS) and ataxia (SARA) rating scales

Background: Reliable movement disorder rating scales are important instruments for accurate interpretation of disease progression from childhood to adulthood. We have previously shown that physiologic movements of healthy children reveal dystonic and ataxic features, resulting in age-related scores of the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS; movement(BFMMS) and disability(BFMDS) subscales) and Scale for the Assessment and Rating of Ataxia (SARA). The Dyskinesia Impairment Scale (DIS, consisting of dystonia (DIS-D) and choreoathetosis (DIS-C) subscales) was recently launched for the quantification of dyskinetic movements in children with cerebral palsy. Until now, it is unclear whether DIS (DIS-D and DIS-C) scores are also influenced by age.

Methods: Three independent movement disorders specialists scored DIS in 52 healthy children (4-16 years, 4 children/year of age; male/female=1), according to previously published methods.(1,2) By regression analysis we determined the association between median DIS scores and age. We compared age-related factors (regression coefficients of z-scores) between DIS, BFMDRS(1) and SARA(2).

Results: DIS total and DIS-D outcomes revealed a significant association with age (β=-0.56; p=0.003 and β=-0.53; p=0.006, respectively), in contrast with DIS-C outcomes (β=-0.18; p=0.381). Comparing age-related factors between DIS-D (B=-0.138), dystonia (BFMMS: B=-0.191 and BFMDS: B=-0.179) and ataxia scales (SARA: B=-0.182) revealed similar regression coefficients (p>0.05), with  the strongest association between DIS-D and BFMMS outcomes (r=0.544; p=0.004).

Conclusions: In healthy children (4-16 years), age influenced dyskinesia, dystonia and ataxia rating scale scores in a similar way. In contrast to the age-dependent DIS-D subscale, DIS-C appeared age-independent, implicating that chorea is not a physiologic feature of neuro-development in children between 4-16 years. DIS-D showed the strongest association with the BFMMS scores, reflecting the same objective (i.e. quantification of dystonic motor output by developing basal ganglia networks). For accurate interpretation of quantitative movement disorder rating scale scores in children, consideration of the age-related effect is advisory.

References: 1. Kuiper MJ, Vrijenhoek L, Brandsma R, Lunsing RJ, Burger H, Eggink H, et al. The Burke-Fahn-Marsden Dystonia Rating Scale is Age-Dependent in   Healthy Children. Mov Disord Clin Pract [Internet]. 2016 Apr [cited 2016 Jul 19]; Available from: http://doi.wiley.com/10.1002/mdc3.12339

2. Brandsma R, Spits AH, Kuiper MJ, Lunsing RJ, Burger H, Kremer HP, et al. Ataxia rating scales are age-dependent in healthy children. Dev Med Child Neurol. 2014;56(6):556–63. 

« Back to 2017 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/comparative-age-relatedness-of-dyskinesia-dystonia-and-ataxia-rating-scales-in-healthy-children/