SLEEP-RELATED HYPERMOTOR (HYPERKINETIC) EPILEPSY (SHE)

OVERVIEW

Sleep-related hypermotor (hyperkinetic) epilepsy (SHE) is a focal epilepsy syndrome with characteristic brief motor seizures occurring from sleep. This syndrome was previously known as nocturnal frontal lobe epilepsy, and (when familial) autosomal dominant nocturnal frontal lobe epilepsy. It has both genetic and structural causes. Patients may have hyperkinetic seizures, or seizures with asymmetric dystonic/tonic motor features. The term sleep-related hyperkinetic epilepsy is to be used when patients have hyperkinetic seizures alone.

Clinical context

This syndrome is characterized by seizure onset in the first 2 decades of life (peak 11-14 years, range 2 months to 64 years). There is a slight male predominance. Antecedent and birth history is typically normal. Head size and neurological examination is normal. Developmental milestones and cognition are typically normal. Intellectual impairment and neuropsychiatric or behavior disorders have been reported in some patients.