[A case of hemimegalencephaly with slowly progressive expansion].
We report a case of a male infant with refractory epilepsy, demonstrating hemimegalencephaly with slowly progressive expansion. The patient experienced his first seizure at 4 months of age. Subsequently, tonic seizures occurred very frequently despite extensive antiepileptic medications, and his development deteriorated. Cranial magnetic resonance imaging (MRI) at 4 months of age showed focal cortical dysplasia in the right opercular area. This focal lesion gradually expanded, and became thickened. Five years later, the dysplastic lesion occupied most of the right cerebral hemisphere and the volume of the right hemisphere increased, indicating hemimegalencephaly. He had profound motor and intellectual retardation. In the abnormal cerebral hemisphere, fluorodeoxyglucose-positron emission tomography (FDG-PET) showed marked hypometabolism, and ictal single photon emission computed tomography (SPECT) showed hyperperfusion, more pronounced in the right frontal area. These findings are consistent with a hemimegalencephaly. Hemimegalencephaly with such a progressive expansion has never been described previously. These findings are consistent with a hemimegalencephaly showing progressive expansion, which has never been described previously.
Yoshioka
Seiichiro
S
Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Kodaira, Tokyo. yoshioka1974830@yahoo.co.jp
Sugai
Kenji
K
Fujikawa
Yoshinao
Y
Komaki
Hirofumi
H
Nakagawa
Eiji
E
Sasaki
Masayuki
M
D002675:Child, Preschool; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D054220:Malformations of Cortical Development / Q000175:diagnosis / Q000503:physiopathology*