Dysplastic Gangliocytoma of the Cerebellum – a case of cerebellar lesion, finally diagnosed five years later

Abstract

Dysplastic Gangliocytoma of the Cerebellum, also called as Lhermitte-Duclos Disease (LDD), is a rare condition. The importance of diagnosing it is to rule out co-existant Cowden's syndrome by thorough clinical examination in view of increased risk of visceral malignancy associated with the latter. Though considered as hamartomatous lesion by some, it is mentioned as a tumour of central nervous system by WHO (Grade I). A lesion, seen in young adults usually, presents with signs & symptoms of raised intracranial tension.We herein describe a casethat came with the symptoms of obstructive hydrocephalus five years back and diagnosed as having some tiny cerebellar lesion on imaging. As the patient was not willing to have biopsy done for definitive diagnosis at that time, it remained undiscovered till present. This time, classical MRI finding (tiger stripes appearance) &diagnostic histopathological features were seen leading to the diagnosis. Association with Cowden's syndrome was not found in this case.