Date of Publication: 28-Feb-2015
A rare cause of hydrocephalus: Lhermitte duclos disease
Author: Mohamed Reda El Ochi, Amal Harmouch, Mustapha Maher, Sanae Sefian
Category: Case Report
Background: Lhermitte Duclos Disease (LDD) is a rare disorder of unknown pathogenesis. It generally affects middle-aged patients and is categorized as a WHO grade 1 tumor. LDD commonly presents with symptoms related to increased intracranial pressure.
Magnetic resonance imaging is the appropriate technique allowing preoperative diagnosis with the characteristic striated pattern of exaggerated folia appearance on T2 weighted image. The principal histological abnormalities are expansion and massive replacement of the internal granular cell layer by large neurons with vesicular nuclei and proeminent nucleoli. Surgery is the treatment of choice. The prognosis is excellent.
Case report: A 41-year-old male was referred to our hospital with loss of consciousness and dizziness lasting for one week. Physical examination revealed left cerebellar signs. In the ophthalmological examination, bilateral papillary oedema was observed. Cranial magnetic resonance imaging revealed a non enhancing striated lesion in the right side of the cerebellum with Hydrocephaly.
A subtotal craniectomy and partial resection of the left cerebellar tumor were performed. The histological and immunohistochemical profile was that of a dysplastic gangliocytoma.
Conclusions: LDD or dysplastic cerebellar gangliocytoma is a rare benign cerebellar mass, probably hamartomatous, with a characteristic aspect magnetic resonance imaging.
Surgery is the only treatment approach that avoids the complications.
Keywords: Lhermitte, Duclos, gangliocytoma, dysplastic.