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.