[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.