Lhermitte-Duclos disease.

A new case of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) was recognized in an adult man after neurosurgery. Conventional, Golgi, and electron-microscopic investigations support the view that this rare lesion results from a progressive hypertrophy of granular cell neurons with excess of myelination of their axons in the molecular layer while inducing the local disappearance of Purkinje cells and the central core of the cerebellar folia. After a follow-up of 4 years, the patient now leads a normal life; no recurrence has occurred and no other neuropathologic alteration have been detected.