Focal polymicrogyria in mother and son

This 9-year-old boy was admitted at the age of 2 with a diagnosis of congen ital hemiparesis while the rest of physical and neurological examination wa s normal. His score in the Wechsler intelligence scale was 80. Right fronto -parietal cortical dysplasia with hemisphere atrophy was evident by compute rized tomography scanning and magnetic resonance imaging. The latter, also disclosed abnormal thick cortex which was interpreted as polymicrogyria or pachygyria. Karyotype was normal. We had a hemifacial motor seizure at the age of 7. At the age of 8 frequent atonic or inhibitory seizures were prese nted. Asymmetric bilateral spike discharges with high voltage in the right hemisphere during the EEG recording were found. His mother, a 35-year-old w oman (Full scale, Adult intelligence scale: 85) also had congenital hemipar esis. She never had seizures and her EEG was normal. Magnetic resonance ima ging disclosed right fronto-parietal cortical dysplasia with ipsilateral he misphere atrophy. Karyotype was normal. Our casts should he interpreted us a familial presentation of the anomaly, probably with autosomal-dominant tr ansmission. (C) 2000 Published by Elsevier Science B.V. All rights reserved .