Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) and its relation to the multiple hamartoma syndrome (Cowden disease).
The clinical manifestations, surgical treatment and postoperative results of three patients with gangliocytomas of the cerebellum (Lhermitte-Duclos disease) are presented. Particular attention is placed in one of the cases, that of a young woman with a short clinical history of episodic symptoms of intracranial hypertension, dizziness and ataxia, with a concomitant frontal meningioma and in the general context of a multiple hamartoma syndrome (Cowden disease). The possible relationship between both diseases is contemplated, since they can be the extremes of a wide spectrum of a peculiar form of phakomatosis.
Service of Neurosurgery, Hospital de Bellvitge, University of Barcelona, Spain.
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J
Rimbau
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F
Isamat
D000328:Adult; D002528:Cerebellar Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D005260:Female; D005729:Ganglioneuroma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006223:Hamartoma Syndrome, Multiple / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed