Tasks for Assessing Dystonia in Young People With Cerebral Palsy

Abstract

Background: Dystonia in childhood is typically associated with cerebral palsy (CP). Dystonia severity scales for CP require prolonged exam protocols with numerous tasks, often making them onerous for routine clinical use. We aimed to identify which individual tasks best approximate dystonia severity compared to the gold standard full protocol.

Methods: In this cross-sectional study, comprehensive exam protocol videos were taken during routine care of ambulatory people with CP age 5 years and up. Five pediatric dystonia experts reviewed individual tasks and the full protocol for dystonia using the Global Dystonia Severity Rating Scale. Experts' written scoring justifications were qualitatively analyzed to determine commonly cited features of dystonia.

Results: When examining the difference in dystonia severity ratings between each task and the full protocol, seated upper extremity tasks had the lowest variance (P < 0.05, F-test) and had lower score differences than the stand/walk/run and seated lower extremity tasks (P < 0.05, repeated measures Friedman test). Experts most commonly identified the following movements as dystonic: wrist flexion (8.4% of all movement statements), finger flexion (7.3%), wrist ulnar deviation (6.8%), toe dorsiflexion (8.4%), ankle inversion (7.9%), and ankle plantarflexion (6.4%). Experts rated dystonic movements as more severe if they were consistently triggered by multiple stimuli (26.8% of all severity statements) or functionally impactful (20.7%).

Conclusions: Seated upper extremity tasks may be valuable for identifying dystonia and estimating its severity during routine clinical care. Clinical dystonia severity assessment could be guided by assessing specific dystonic movements, the consistency with which they are triggered, and their functional impact.

Keywords: Cerebral palsy; Dystonia; Motor exam; Pediatric movement disorders.