Acute Hydrocephalus Revealing Unusual Cerebellar Mass: Dysplastic Cerebellar Gangliocytoma or Lhermitte-Duclos Disease (LDD)

摘要

Lhermitte-Duclos disease (LDD) or dysplastic gangliocytoma of the cerebellum is a rare benign lesion of uncertain pathogenesis characterized by overgrowth of cerebellar ganglion cells which replace granular cells and Purkinje cells; this results in gross thickening of the cerebellar folia. It is revealed by symptoms of raised intracranial pressure, cerebellar impairment and obstructive hydrocephalus. We reported the case of a 41-year-old male who presented with symptoms of acute raised intracranial pressure. Brain computed tomography (CT) scan revealed hydrocephalus due to compression of the 4th ventricle by a large non-enhancing left cerebellar mass. Magnetic resonance imaging (MRI) showed a space-occupying lesion within the left cerebellar hemisphere with unusual striation. Radical surgery was retained though the margins with normal cerebellar tissue were not distinct. Clinical complications after gross total or partial removal of Lhermitte-Duclos lesions have been rarely reported in the literature; herein we stress the importance of extreme caution in removing these lesions in cerebellar areas that have no distinct margins between the lesion and normal tissue.