Cerebellar gangliocytoma dysplasticum in an infant

A boy, 25 months old, presented a cerebellar syndrome with increased intracranial pressure. Despite a ventriculo-peritoneal bypass, death occurred at the age of 4 1/2 years. Autopsy revealed a very large cerebellum, presenting a "glossy" appearance, with swelling of the brain stem. Histologically, there was a very unusual lesion with a diffuse disorganization of the cortex, a neuronoglial mixed proliferation, a large number of calcospherites, small tumour-like nodules and many leptomeningeal glial bridges. The authors consider that their case is not related to L'Hermitte-Duclo's disease, nor to gangliogliomas, and they discuss the hypothesis of hamartoma.