Lhermitte-duclos Disease in a Young Adult Case Report

Dysplastic gangliocytoma of the cerebellum is a very rare lesion. The other names assigned to this pathology are Lhermitte-Duclos disease. ganglioneuroma. hamartoma of the cerebellum. purkengioma. granule cell hypertrophy or granulomolecular hypertrophy of the cerebellum. Only few cases have been reported in the literature (1-20).since the first report by Lhermitte and Duclos (11).Clinically. the disease appears most often in young adults as a slowly expanding lesion of the posterior fossa. specifically originating from the cerebeller hemispheres. The pathophysiology of the disease is poorly understood. and this accounts for the multiple names assigned to the entity (1.7). The most important point is that the diagnosis has never been suspected preoperatively in all published cases. In this paper. we present a case of LhermitteDuclos disease with an unusual vermian localization. Our purpose is to discuss the clinical and radiological aspects of the disease that could support a preoperative diagnosis.