[Cerebral complications of incontinentia pigmenti. A clinicopathological study of a case]

Abstract

A baby born with the typical features of incontinentia pigmenti, developed convulsions from the 5th day onward. CSF was normal. CT scan showed diffuse hypodensities in the left hemisphere. The baby died at 2 months of age of primary pulmonary hypertension. The neuropathological examination showed a normal morpho-histogenesis of the brain. There were necrotic, non inflammatory changes in the cortex and white matter, which did not affect a vascular topography. The lesions appeared to have occurred in the neonatal period and were markedly predominating in the left hemisphere.