WEST SYNDROME DUE TO PERINATAL INSULTS

Clinical, neuroradiological data and outcome of epilepsy of 32 patient s with symptomatic infantile spasms caused by perinatal insults are re ported. Neuroradiological investigations showed focal as well as diffu se cerebral lesions. Fifteen children had a porencephalic lesion, 12 h ad periventricular leukomalacia and five had diffuse bilateral cerebra l atrophy, associated with status marmoratus in one child and bilatera l ulegyria in another. Eight patients (25%) had epilepsy following Wes t syndrome. In the group of 15 children with porencephalic cysts, the lesion was always unilateral, but topography and extension varied. Eig ht children had circumscribed cysts involving the rolandic or parieto- occipital regions, and the outcome of epilepsy was favorable. Seven pa tients had large lesions involving the frontal lobe, associated with u nilateral hemispheric atrophy in four cases. These four children had a n unfavorable epilepsy outcome. In the 12 patients with periventricula r leukomalacia, all premature, eleven had no seizure relapse after the spasms; one of these children had epilepsy. In the five full-term pat ients with bilateral and diffuse cerebral lesions, three children had epilepsy at the last observation. In children with leukomalacia and in patients with localized porencephalic lesions the outcome of epilepsy appears to be better than in patients with diffuse cerebral lesions o r in children with extensive porencephalic cysts, particularly those i nvolving the frontal lobe.