Hemiconvulsion-hemiplegia-epilepsy syndrome: characteristic early magnetic resonance imaging findings.
We report three patients with hemiconvulsion-hemiplegia-epilepsy syndrome who presented acutely and were shown to have striking neuroimaging findings suggestive of diffuse cytotoxic edema confined to one hemisphere, including extensive diffusion-weighted imaging abnormalities in two cases. Two patients subsequently developed progressive and extensive atrophy of the involved hemisphere. These findings are consistent with earlier descriptions of the classic neuroradiologic features of this syndrome and are helpful in the differential diagnosis of acute infantile hemiplegia. Further, the findings support the previously proposed pathogenetic mechanism of neuronal injury caused by status epilepticus.
Freeman
Jeremy L
JL
Department of Neurology, Royal Children's Hospital, Parkville VIC, Australia. fremanj@cryptic.rch.unimelb.edu.au
Coleman
Lee T
LT
Smith
Lindsay J
LJ
Shield
Lloyd K
LK
D001224:Aspartic Acid / Q000031:analogs & derivatives* / Q000378:metabolism; D001921:Brain / Q000473:pathology; D001929:Brain Edema / Q000175:diagnosis; D002675:Child, Preschool; D004292:Dominance, Cerebral / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006429:Hemiplegia / Q000175:diagnosis*; D006801:Humans; D007089:Image Enhancement; D007223:Infant; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D003294:Seizures, Febrile / Q000175:diagnosis*
D001224:Aspartic Acid; C000179:N-acetylaspartate