[Benign intracranial hypertension and Marchiafava-Micheli disease].
A 35 year-old caucasian man suffered from paroxysmal nocturnal haemoglobinuria (PNH) or Marchiafava-Micheli's disease diagnosed in 1976 and complicated by several thrombotic episodes. He developed a benign intracranial hypertension. A digitalized intravenous angiography showed occlusion of both lateral sinuses. Partial improvement followed lombo-peritoneal shunting and steroid therapy. Cerebral venous thrombosis is a well-known complication of PNH but only a few cases have been radiologically and/or pathologically proven. It usually involves the superior longitudinal sinus and/or cortical veins resulting in hemorrhagic infarction of poor outcome. Benign intracranial hypertension due to a venous occlusion is rare. In 3 published cases, as in our own, the neurologic outcome was good. Steroid therapy seems useful. The risks of anticoagulant therapy are discussed.
D000328:Adult; D003392:Cranial Sinuses; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006801:Humans; D002542:Intracranial Embolism and Thrombosis / Q000209:etiology*; D008297:Male; D010690:Phlebography; D011559:Pseudotumor Cerebri / Q000209:etiology*
Hypertension intrâcranienne bénigne et maladie de Marchiafava-Micheli.