Bilateral perisylvian polymicrogyria in three generations

A family is described in which bilateral perisylvian polymicrogyria was pre sent in 6 members of 3 conservative generations. Typical anatomic and clini cal findings 6 of the syndrome, with a mild phenotype, were present in the 5 affected women from all 3 generations. More severe impairment was observe d in the only affected male individual, a boy, in the third generation. Ana lysis of the pedigree and severity of the phenotype in the affected boy are consistent with transmission of an X-linked dominant trait, although other patterns of inheritance cannot be ruled out with certainty.