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.
D001344:Autopsy; D002528:Cerebellar Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D002557:Cerebrospinal Fluid Shunts; D002675:Child, Preschool; D005729:Ganglioneuroma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006222:Hamartoma / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007427:Intracranial Pressure; D008297:Male