SNEDDONS SYNDROME - A SERIES OF 9 CASES

We observed a series of 9 patients (1 male, 8 females, mean age 49 yea rs) 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 ev ents. Angiography of the brain revealed multiple distal arterial occlu sions in 5 cases and a moya-moya type collateral network in 2. Positiv ity for anticardiolipid antibodies fluctuated in 4 cases and there was a lupic syndrome in 2. Systemic lupus erythemosus was diagnosed in th e last patient. Mitral valve defects were seen in 5 patients, includin g 3 due to post-rhematitis sequelae which became symptomatic before th e appearance of signs of neurocutaneous involvement. Among these three patients, laboratory tests revealed a lupus band in one, anticardilip id antibodies in another and obliterating fibrous endartiritis of the renal arteries in the third. Sneddon's syndrome presents with heteroge neous signs related to its complex pathophysiology.