Despite dystonia: natural history of delayed-onset pediatric secondary dystonia.

ABSTRACT

Background: Dystonia is a movement disorder involving involuntary movements and/or postures of the limbs, trunk, neck or face. Secondary dystonia following brain injury is uncommon, with unfavorable long-term consequences. Given the limited evidence regarding pediatric secondary dystonia, this study's aim was to document the natural history of the condition and the effect of interventions on its symptomatology. Methods: We describe three cases of girls (age 8 to 11 y) who developed dystonia secondary to an acquired brain injury, received intensive rehabilitation treatments and were followed for 8-33 months post-injury. In all three cases, secondary dystonia appeared 1-3 months post-insult. Results: In none of the cases was there alleviation of dystonic symptoms over time despite physical and pharmacological interventions; in two cases the dystonic hand is now used as an assisting hand only, whereas in the third it is completely non-functional. However, despite their impairment, two girls achieved basic functional independence and one is partly independent in activities of daily living. Conclusions: Rehabilitation professionals who work with pediatric patients susceptible to developing secondary dystonia should be aware of its possible consequences and inform families and staff. Intensive task-specific training during rehabilitation may be key to regaining overall functional capabilities despite residual impairment.