Unusual Magnetic Resonance and Neuropathological Findings in Hemimegalencephaly: Report of a Case Following Hemispherectomy

Abstract

Hemimegalencephaly is a rare disorder manifest by early epilepsy, mental retardation and hemiplegia. The neuropathology has been described in only 15 cases to date. The present case provides a further description of the pathology in a hemispherectomy specimen and shows unusual features, including cystic breakdown of the white matter possibly related to the long duration of the features, including cystic breakdown of the white matter possibly related to the long duration of the disease; the subject was 13 years older than previously documented cases. MRI findings were also unusual in showing mass effect and ventricular compression in the affected hemisphere, features not previously described in hemimegalencephaly.