Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease).
The case of a 30-year-old male patient is presented who was admitted with a 3-month history of elevated intracranial pressure and reduced-hearing on the right. CT revealed hydrocephalus due to displacement of the 4th ventricle by a large non-enhancing cerebellar mass with calcifications. MRI sequences showed a space-occupying lesion within the right cerebellar hemisphere with unusual septations. At operation, the poorly demarcated lesion was excised from the apparently normal surrounding cerebellar tissue and histologically proven to be a dysplastic gangliocytoma (Lhermitte-Duclos disease). Clinical and neuroradiological appearance of this rare entity, as well as hypotheses on its pathogenesis are discussed.
Rainov
N G
NG
Department of Neurosurgery, Faculty of Medicine, Martin-Luther-University, Halle (Saale), Germany.
Holzhausen
H J
HJ
Burkert
W
W
D000328:Adult; D002528:Cerebellar Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D002531:Cerebellum / Q000473:pathology*; D003051:Cochlea / Q000503:physiopathology; D005729:Ganglioneuroma / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D006223:Hamartoma Syndrome, Multiple / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D006311:Hearing Disorders / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D006849:Hydrocephalus / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D007427:Intracranial Pressure; D008279:Magnetic Resonance Imaging; D008297:Male; D014057:Tomography, X-Ray Computed