TITLE:
Acute Hydrocephalus Revealing Unusual Cerebellar Mass: Dysplastic Cerebellar Gangliocytoma or Lhermitte-Duclos Disease (LDD)
AUTHORS:
C. Karekezi, M. Boutarbouch, O. Coulibaly, S. Derraz, A. El Ouahabi, A. El Khamlichi
KEYWORDS:
Lhermitte-Duclos Disease; Dysplastic Gangliocytoma; MRI; Cerebellar Tiger Stripes
JOURNAL NAME:
Neuroscience and Medicine,
Vol.4 No.3,
September
11,
2013
ABSTRACT: 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.