Neuropathologic features, including pontine schwannosis, in a four-year survivor of probable Reye's syndrome with secondary Möbius syndrome.

Abstract

Frequent seizures and loss of motor, language, and intellectual skills necessitated care in an institution for the physically and mentally handicapped for 4 years after diagnosis of presumed Reye's syndrome in a 10-month-old boy. M obius syndrome was diagnosed and bilateral tarsorrhaphy performed for exposure keratitis. Postmortem examination revealed multiple old boundary zone infarcts in the cerebral cortex, small cavitated infarcts in basal ganglia and thalami, diffuse neuronal loss and gliosis, and a focus of old necrosis in the pons; brain stem herniation during the acute phase of Reye's syndrome was responsible for the latter lesion. Irregular congeries of proliferated peripheral nerves, confirmed by ultrastructural study, were ramifying in the gliotic pontine tegmentum. The pathologic substrate for the neurovegetative state in a long-term survivor of probable Reye's syndrome appears to be multifocal cerebral infarction.