Dysplastic cerebellar gangliocytoma (Lhermite-Duclos disease): a rare entity and review of literature

Abstract

Lhermite-Duclos disease (LDD) is a distinct entity of the cerebellum by its unique pathological and radiological findings, and strong association with CS. The exact nature of this lesion is unclear, but it has been considered as a hypertrophy, a hamartoma, or a benign neoplasm. The terminologies mentioned previously include hamartoma of the cerebellum, hamartoblastoma, granule cell hypertrophy, diffuse ganglioneuroma of the cerebellar cortex, gangliocytoma myelinicum, myelinated neurocytoma, and Purkinjeoma.4 Most cases are reported in adults and PTEN mutations have been identified in virtually all cases of adult-onset disease.5 The most common clinical presentation is dysmetria. The other signs include signs and symptoms of mass effect including cranial nerve deficits and seizures.6 MRI is a sensitive investigation which reveals enlarged folia with alternating T1hypointense and T-2 hyperintense tiger-striped striations, which do not enhance.7