Benign intracranial hypertension and Marchiafava-Micheli disease
Benoit, P.; Lozes, G.; Destee, A.; Jouet, J.P.; Jomin, M.; Warot, P.
Revue Neurologique (Paris) 142(10): 782-785
1986
ISSN/ISBN: 0035-3787 PMID: 3823710 Document Number: 280341
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 rate. In 3 published cases, as in our own, the neurologic outcome was good. Steroid therapy seems useful. The risks of anticoagulant therapy are discussed.