DYSPLASTIC GANGLIOCYTOMA OF THE CEREBELLUM (LHERMITTE-DUCLOS SYNDROME) : A CASE REPORT

Lhermitte-Duclos disease or dysplastic cerebellar gangliocytoma is a rare entity characterized by a hamartomatous lesion in the posterior fossa. Cowden's syndrome or hamartoma-neoplasia syndrome is a rare underdiagnosed autosomal dominant genodermatosis with a high incidence of malignant tumors. We report the case of a 42-year-old man presented with a cerebellar syndrome, Magnetic resonance (MR) imaging of the brain was performed and revealed a cerebellar left lesion suggestive of a Lhermitte-Duclos disease. At operation, a demarcated lesion was excised from the apparently normal surrounding cerebellar tissue and proved histologically to be Lhermitte-Duclos disease.