Angelman Syndrome

Angelman syndrome (AS) is a neurodevelopmental disorder characterized by intellectual disability (ID), limited expressive language, epilepsy, motor impairment, and a behavioral profile notable for strong social affinity and excitability. It is caused by insufficient expression of ubiquitin-protein ligase E3A from the UBE3A gene on the maternal copy of chromosome 15. Common clinical concerns include epilepsy, disordered sleep, and behavioral challenges such as hypermotoric behavior, aggression, and self-injury. This chapter reviews the evidence for interfering behaviors across the lifespan in patients with AS and the approach to evaluation, treatment including pharmacology, and school-based accommodations. Research supporting the efficacy of these interventions is presented when available.