Stimulant intolerance in children with Angelman syndrome with hyperactivity: a case series.

Abstract

Angelman syndrome is a neurogenetic disorder resulting from the loss of expression of the ubiquitin-protein ligase E3A gene on chromosome 15. Problematic behaviors including attention-deficit/hyperactivity disorder (ADHD) symptoms of hyperactivity, impulsivity and inattention are highly prevalent in Angelman syndrome. The efficacy, safety and tolerability of stimulant medications in children with Angelman syndrome for the treatment of ADHD symptoms have not been previously reported. We describe three boys with Angelman syndrome who were treated with open-label stimulant medications for ADHD symptoms. Stimulant medications were highly intolerable, and treatment had to be discontinued after limited dosing in all three cases due to marked increases in hyperactivity and impulsivity along with worsened distractibility. The findings of this study suggest that stimulant medications may be ineffective and poorly tolerated in children with Angelman syndrome.