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[Acute benign cerebral angiopathy. 6 cases].

The 6 cases reported here constitute, with 5 previously published cases, a special nosological entity tentatively called "acute benign cerebral angiopathy" by the authors. These 11 cases have in common certain radiological and clinical features. Arteriography shows segmental, multifocal and assymetrical stenoses involving the cerebral arteries between Willis' circle and the terminal arterioles and looking like "strings of sausages". The lesions disappear within one month and present the radiological characteristics of arteritis of medium caliber vessels. The clinical symptoms are suggestive of meningeal haemorrhage or acute cerebromeningeal oedema, with acute repetitive attacks of severe headache and agitation with obnubilation; epileptic seizures and transient neurological deficit may occur. True meningeal haemorrhage confirmed by lumbar puncture is seen in nearly one half of the cases; it seems to be due to alterations in the blood-brain barrier induced by the angiopathy. Intracerebral haematoma may develop, but the disease is usually benign and regresses spontaneously in a few days. None of the usual causes of cerebral arteritis (intra-cranial infection, collagen disease, allergic or toxic angitis) has been found. Pseudo-arteritis (notably spasm of ruptured arterial aneurysms) has been excluded. No aetiological factor common to the 11 cases reported has been elicited, although 6 of the patients had recently given birth and our 6 patients had benign virus infection before or during the clinical manifestations of the disease. In the authors' opinion, the most rewarding line of research would be the role of short acute attacks of arterial hypertension.