A case of congenital bilateral perisylvian syndrome due to bilateral schizencephaly.
A case of congenital bilateral perisylvian syndrome (CBPS) associated with bilateral perisylvian schizencephaly in a 24-year-old woman is reported. She presented the classical clinical triad of CBPS, which included congenital facio-masticatory diplegia, epilepsy and only mild mental retardation, despite the presence of bilateral, open-lip clefts in the perisylvian region. We hypothesize that the minimal loss of cortical tissue, along with the possible sparing of vital white matter association fibers and neuronal plasticity might have contributed to the better functional outcome in this patient.
Vinayan
K Puthenveettil
KP
National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Aoi-ku, Shizuoka, Japan. vinayankp@aims.amrita.edu
Terada
Kiyohito
K
D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000473:pathology; D000328:Adult; D001921:Brain / Q000002:abnormalities* / Q000473:pathology; D001927:Brain Diseases / Q000151:congenital / Q000175:diagnosis; D002540:Cerebral Cortex / Q000002:abnormalities / Q000473:pathology; D004569:Electroencephalography / Q000706:statistics & numerical data; D005260:Female; D007839:Functional Laterality; D006801:Humans; D008279:Magnetic Resonance Imaging; D009421:Nervous System Malformations / Q000473:pathology