MICROLISSENCEPHALY - A HETEROGENEOUS MALFORMATION OF CORTICAL DEVELOPMENT

We report the neonatal courses, early postnatal development, and neuro imaging findings of 17 patients with marked microcephaly and simplifie d cerebral gyral patterns, a condition that we call microlissencephaly . Retrospective analyses of the clinicoradiologic features of these pa tients allowed segregation of the patients into 5 distinct groups with varying outcomes. The apparent discreteness of these groups suggests multiple etiologies of this malformation, although there appears to be a strong genetic component with probable autosomal recessive inherita nce. Utilizing the neonatal course and neuroradiologic features of the se infants allows classification of specific subsets, which may be use ful to predict outcome.