Sa. Renowden et M. Squier, UNUSUAL MAGNETIC-RESONANCE AND NEUROPATHOLOGICAL FINDINGS IN HEMIMEGALENCEPHALY - REPORT OF A CASE FOLLOWING HEMISPHERECTOMY, Developmental Medicine and Child Neurology, 36(4), 1994, pp. 357-361
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.