Article ID Journal Published Year Pages File Type
5628535 Epilepsy & Behavior Case Reports 2017 4 Pages PDF
Abstract

•Hemimegalencephaly is a rare disorder of neuronal migration characterized by epilepsy, developmental delay and hemiparesis.•Outcomes of hemimegalencephaly are generally poor; however, this is typically reported in pediatric populations, not adults.•Hemispherectomy is beneficial in reducing seizure burden; however, most do not obtain seizure-freedom.•Seizures are typically focal onset; however, the epileptogenic area may increase with poor seizure control.

Hemimegalencephaly (HME) is a rare disorder of cortical development with overgrowth of one cerebral hemisphere. Patients have intellectual delay, hemiparesis and severe epilepsy. Drug-resistant epilepsy is often treated with a hemispherectomy. We review the literature on HME natural history and report a 26-year-old man with HME who did not undergo hemispherectomy in childhood with recurrent focal convulsive or non-convulsive status epilepticus.Few patients with HME have been followed into adulthood. Reported adult cases have milder epilepsy or underwent hemispherectomy in childhood. Patients surviving to adulthood have poor outcomes, regardless of treatment method, although seizure burden is improved with hemispherectomy.

Related Topics
Life Sciences Neuroscience Behavioral Neuroscience
Authors
, ,