[Sneddon syndrome: 9 cases]

We observed a series of 9 patients (1 male, 8 females, mean age 49 years) who had experienced cerebral vascular events with livedo racemosa (Sneddon's syndrome). Vascular dementia occurred in 3 patients and in the 6 others there was a single or several acute cerebral ischaemic events. Angiography of the brain revealed multiple distal arterial occlusions in 5 cases and a moya-moya type collateral network in 2. Positivity for anticardiolipid antibodies fluctuated in 4 cases and there was a lupic syndrome in 2. Systemic lupus erythemosus was diagnosed in the last patient. Mitral valve defects were seen in 5 patients, including 3 due to post-rhematitis sequelae which became symptomatic before the appearance of signs of neurocutaneous involvement. Among these three patients, laboratory tests revealed a lupus band in one, anticardilipid antibodies in another and obliterating fibrous endartiritis of the renal arteries in the third. Sneddon's syndrome presents with heterogeneous signs related to its complex pathophysiology.