[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.
Antoine
J C
JC
Service de Neurologie, Hôpital de Bellevue, Saint-Etienne.
Michel
D
D
Garnier
P
P
Absi
L
L
Boucheron
S
S
Barral
F G
FG
Laurent
B
B
D017153:Antibodies, Anticardiolipin / Q000302:isolation & purification; D002545:Brain Ischemia / Q000209:etiology*; D002560:Cerebrovascular Circulation; D002561:Cerebrovascular Disorders / Q000209:etiology*; D015140:Dementia, Vascular / Q000209:etiology*; D005260:Female; D006349:Heart Valve Diseases / Q000150:complications; D006801:Humans; D008297:Male; D008875:Middle Aged; D017445:Skin Diseases, Vascular / Q000150:complications* / Q000473:pathology; D013577:Syndrome
D017153:Antibodies, Anticardiolipin
Syndrome de Sneddon: 9 cas.