Sneddon Syndrome without Antiphospholipid Antibodies in 55 Cases: Clinical, Radiological and Pathophysiological Features (S43.005)

Abstract

Objective: To study the pathophysiological mechanisms of ischemic strokes (IS) and the prognosis of a cohort of patients with a Sneddon syndrome without antiphospholipid antibodies (SSAPL-). Background SS is characterized by the association of a livedo reticularis (LR) with strokes. The clinical and radiological features of the neurological manifestations, its prognosis and the frequency of associated cardiac injury remain poorly known, particularly in the absence of APL. Design/Methods: Review of clinical data and brain imaging in 55 consecutive patients (83% women) with SSAPL-, followed up at our institution between 1991 and 2011. Results: LR was first noticed at a mean age of 32 years and the first stroke at 43; 53% had hypertension. Seventy-four strokes were reported, including 76% of IS, 15% of TIA and 9% of hemorrhagic strokes. A mitral or aortic valvulopathy was found in 52% of the 42 patients with an echocardiography. Brain imaging showed 177 IS of 3 different types: large territorial (43%), small distal cortical subcortical (14%) and lacunar (23%) IS; 70% of the IS were located in the superficial territory of the middle cerebral artery. No significant link was found between the valvulopathy and the presence of territorial IS. Other brain abnormalities were frequent: cortical and subcortical atrophy (>90%) and slight leucopathy. After a mean follow-up of 7.4 years, 82% of patients had a modified Rankin score ≤2. Global IS recurrence rate was 12% with a similar annual rate in the antiplatelet group (1.6%) compared to the anticoagulation group (1.5%). Conclusions: SSAPL- is not only a neuro-cutaneous disorder, frequently involving a valvulopathy. This one does not influence the IS type suggesting IS being caused by the small and medium cerebral arteries vasculopathy. Our results show no progression to a serious handicap in the majority of cases and a small recurrence rate under antiplatelet therapy. Disclosure: Dr. Bottin has nothing to disclose. Dr. Frances has nothing to disclose. Dr. De Zuttere has nothing to disclose. Dr. Boelle has nothing to disclose. Dr. Muresan has nothing to disclose. Dr. Alamowitch has nothing to disclose.