PERIVENTRICULAR HETEROTOPIA AND EPILEPSY

We report a family with nodular subependymal masses of heterotopic gra y matter occurring in six members in four generations. Only female mem bers of the family are affected, and there is a high rate of spontaneo us abortion, consistent with X-linked dominant inheritance, and lack o f viability in affected males. Both in this family and in sporadic cas es of subependymal heterotopias there is a high frequency of convulsiv e disorders, suggesting that epilepsy may be the major clinical manife station of this developmental defect.