UNUSUAL MAGNETIC-RESONANCE AND NEUROPATHOLOGICAL FINDINGS IN HEMIMEGALENCEPHALY - REPORT OF A CASE FOLLOWING HEMISPHERECTOMY

Hemimegalencephaly is a rare disorder manifest by early epilepsy, ment al retardation and hemiplegia. The neuropathology has been described i n only 15 cases to date. The present case provides a further descripti on of the pathology in a hemispherectomy specimen and shows unusual fe atures, including cystic breakdown of the white matter possibly relate d to the long duration of the disease; the subject was 13 years older than previously documented cases. MRI findings were also unusual in sh owing mass effect and ventricular compression in the affected hemisphe re, features not previously described in hemimegalencephaly.