Therapy of Sneddon Syndrome

Abstract

We report the case of a young woman with progressive cognitive decline and epilepsy. She showed ischemic cerebrovascular disease and proximal livedo racemosa. Antiphospholipid antibody (aPL) could not be detected and there were no microemboli on continuous transcranial Doppler ultrasonography monitoring. Histology of cerebral vessels showed intimal hyperplasia in small leptomeningeal venous vessels and micronecrosis of grey and white matter. We subsequently made the diagnosis of aPL-negative Sneddon Syndrome (SNS). Anticoagulation with warfarin could not be initiated because of a drug-resistant epilepsy with the risk of falls and subsequent bleeding; immunosuppression with steroids and azathioprine was ineffective, as was initial antiplatelet therapy with clopidogrel alone. However, when we intensified antiplatelet therapy by combining clopidogrel and ASS, a slowing of disease progression, as assessed by neuropsychological testing and magnetic resonance imaging, was noted on a follow-up after 6 months. Therapeutic options in SNS in both aPL-positive and aPL-negative patients with SNS are discussed.

The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa

“…The syndrome usually occurs in young adults, and more often in women (80% of cases) [46, 55, 106]. The mean age of patients at time of first clinical cerebral ischaemic event is typically before the age of 45 years (mean 38.6 years with a range of 16 to 58 years) [28,29]. A similar combination of strokelike episodes and livedo racemosa has been reported to occur in children in rare circumstances [105].…”

“…The prevalence of antiphospholipid antibodies (aplAB) in SS has had widely differing figures reported, ranging from 0% to 85% depending upon the series [28,29,45]. Most authors suggest that 40-50% of SS patients are aplAB-positive [43,46].…”

“…However, categorisation of SS patients into two subsets (aplABpositive and aplAB-negative) might influence disease management. Flöel et al stressed that aplAB-positive patients might be treated like primary antiphospholipid syndrome [28]. A retrospective analysis of patients with primary antiphospholipid syndrome suggested that long-term anticoagulation is advisable [49].…”

“…A retrospective analysis of patients with primary antiphospholipid syndrome suggested that long-term anticoagulation is advisable [49]. Therefore, in aplAB-positive SS patients, high dose warfarin is the most common therapy used [28]. In aplAB-positive patients, warfarin seems to be more effective than aspirin.…”

“…Prostaglandin E1 might improve microcirculation by altering the rheological properties of the blood. It has been reported that five patients treated with this triple combination showed no further deterioration over the course of 3-5 years [28,106,107]. Additionally, it is necessary to avoid smoking and oestrogencontaining oral contraceptives [106,107].…”