[Tumoral forms of tuberous sclerosis. The clinical and therapeutic importance of concomitant hydrocephalus (author's transl)]

Abstract

In a young man and a pregnant young woman presenting since childhood of tuberous sclerosis without epilepsy or mental disorders severe intracranial hypertension developped secondary to obstructive hydrocephalus. Ventriculo-peritoneal shunting unilateral in the first case, bilateral in the second, releaved the patients of all their symptoms. Angiographic studies in both cases showed the existence of extracerebral visceral lesions. After a short review of similar published cases and the proposed treatments the authors suggest as only treatment a shunting procedure without attempt of removal of intracerebral nodules.