Clinical patterns of dystonia and choreoathetosis in participants with dyskinetic cerebral palsy

Dev Med Child Neurol. 2016 Feb;58(2):138-44. doi: 10.1111/dmcn.12846. Epub 2015 Jul 15.

Abstract

Aim: The aim of the study was to map clinical patterns of dystonia and choreoathetosis and to assess the relation between functional classifications and basal ganglia and thalamus lesions in participants with dyskinetic cerebral palsy (CP).

Methods: In this cross-sectional study, 55 participants with dyskinetic CP (mean age 14y 6mo, SD 4y 1mo; range 6-22y) were assessed with the Dyskinesia Impairment Scale and classified with the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), and Communication Function Classification System (CFCS).

Results: Dystonia and choreoathetosis are simultaneously present. Median levels of dystonia (70.2%) were significantly higher than levels of choreoathetosis (26.7%) and both were significantly higher during activity than at rest (both p<0.01). High correlations were found between dystonia levels and GMFCS level (Spearman's rank correlation coefficient, rS =0.70; 95% confidence interval [CI] 0.53-0.81; p<0.01) and MACS (rS =0.65; 95% CI 0.47-0.81; p<0.01), and fair correlation with CFCS (rs =0.36; 95% CI=0.11-0.57; p<0.05). No significant correlation was found between choreoathetosis levels and motor classifications. Finally, higher choreoathetosis levels were found in participants with pure thalamus and basal ganglia lesions (p=0.03) than mixed lesions, but not for dystonia (p=0.41).

Interpretation: Dystonia and choreoathetosis increase during activity. However, dystonia predominates and seems to have a larger impact on functional abilities. Our findings further suggest that choreoathetosis seems to be more linked to pure thalamus and basal ganglia lesions than dystonia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Athetosis / epidemiology
  • Athetosis / etiology
  • Athetosis / physiopathology*
  • Basal Ganglia Diseases / pathology
  • Cerebral Palsy / complications
  • Cerebral Palsy / epidemiology
  • Cerebral Palsy / physiopathology*
  • Child
  • Chorea / epidemiology
  • Chorea / etiology
  • Chorea / physiopathology*
  • Cross-Sectional Studies
  • Dystonia / epidemiology
  • Dystonia / etiology
  • Dystonia / physiopathology*
  • Female
  • Humans
  • Male
  • Severity of Illness Index
  • Thalamus / pathology
  • Young Adult