title: Strokes in Sneddon syndrome without antiphospholipid antibodies.
abstract: OBJECTIVE
  Sneddon syndrome (SS) is characterized by the association of a livedo reticularis with stroke. Clinicoradiological features of its neurological manifestations, its prognosis, and the frequency of associated cardiac valvulopathy remain poorly known, particularly in the absence of antiphospholipid antibodies (APL). The objectives were to assess the clinicoradiological pattern of SS without APL (SSAPL- ) and its midterm prognosis.
  
  
  METHODS
  Clinical data, transthoracic echocardiograms, and brain imaging of 53 consecutive patients (83% women) with SSAPL- , followed up at our institution between 1991 and 2011, were reviewed.
  
  
  RESULTS
  Seventy-four strokes were reported; 76% were ischemic strokes (IS), 15% transient ischemic attacks, and 9% hemorrhagic strokes. Heart valve lesions were found in 50% of the cases. Brain imaging showed 177 IS of 3 different types: large territorial (43%), small distal corticosubcortical (14%), and small deep (23%) IS. No significant association was found between the valve involvement and the presence of territorial IS. After a mean follow-up of 7.4 years, 82% of patients had a modified Rankin Scale score ≤ 2. The ischemic event recurrence rate was 20%, with a similar annual rate in the antiplatelet group (3%) compared to the anticoagulation group (2.7%).
  
  
  CONCLUSIONS
  SSAPL- is not only a neurocutaneous disorder, but is frequently associated with heart valve involvement. The latter does not influence the IS type, which suggests that strokes are caused by vasculopathy of the small and medium-size cerebral arteries. Our results show no progression toward a serious disability in the majority of the cases and a moderate recurrence rate under antiplatelet therapy.
authors: Department of Neurology, Saint-Antoine University Hospital, Paris.
authors: Laure
authors: L
authors: Bottin
authors: Camille
authors: C
authors: Francès
authors: Dominique
authors: D
authors: de Zuttere
authors: Pierre-Yves
authors: PY
authors: Boëlle
authors: Ioan-Paul
authors: IP
authors: Muresan
authors: Sonia
authors: S
authors: Alamowitch
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D017152:Antibodies, Antiphospholipid / Q000097:blood*; D001921:Brain / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012307:Risk Factors; D018860:Sneddon Syndrome / Q000097:blood* / Q000000981:diagnostic imaging*; D020521:Stroke / Q000097:blood* / Q000000981:diagnostic imaging*; D055815:Young Adult
chemical_list: D017152:Antibodies, Antiphospholipid