DYSPLASTIC GANGLIOCYTOMA IN PEADIATRIC AGE GROUP WITH ACUTE PRESENTATION

Abstract

Abstract: Dysplastic gangliocytoma (Lhermitte-Duclos disease, LDD) is a rare hamartomatous lesion of the cerebellum, characterized by thickening of the molecular and granular cell layers. We present a case of a 5-year-old boy with LDD, presenting with headache, vomiting, and fever. Imaging revealed a hypodense lesion in the left cerebellar hemisphere causing mass effect and hydrocephalus. MRI depicted abnormal signal intensity and characteristic enhancement patterns with tiger stipe appearance. Surgical intervention with ventriculoperitoneal shunt placement resulted in symptom resolution. LDD's natural history and management remain unclear, warranting further investigation in pediatric cases. This case highlights the importance of considering LDD in pediatric patients presenting with cerebellar lesions.

Key words: Dysplastic gangliocytoma, Lhermitte-Duclos disease, hydrocephalus, tiger stripe.