FAMILIAL SCHIZENCEPHALY

Schizencephaly is a brain malformation characterized by infolding of c ortical gray matter along a hemispheric cleft near the primary cerebra l fissures. Although the etiology is unknown, genetic counseling has n ot been advocated because of its sporadic occurrence. We describe a fa mily with two affected siblings. Both cases were characterized by hemi paresis, lack of gestational or postnatal complications, and diagnosti c radiologic findings. We mise the possibility of a genetic etiology i n some cases of schizencephaly and suggest a re-examination of the nee d for genetic counseling.