Lhermitte-Duclos disease associated with meningioma - a rare case

Abstract

Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum is a benign disease, extremely rare, characterized by loss of normal cerebellar cortical architecture and focal thickening of the folia. Lhermitte-Duclos disease as a major CNS manifestation of Cowden disease is a rare condition. The association between Lhermitte-Duclos disease and meningioma is even more rare, only few cases having been documented in the literature. We present a very rare case of a 53-year-old woman who was admitted in our department for recurrent episodes of headache and epilepsy. Magnetic resonance imaging revealed the presence of a well-defined lesion with an abnormal laminated pattern of cortical architecture involving most of the right cerebellar hemisphere, compressing the fourth ventricle and also a meningioma. Complete removal of the meningioma and partial removal of the cerebellar lesion was performed and histopathological exam confirmed Lhermitte-Duclos disease.