ABSTRACT

The syndrome is predominantly associated with systemic lupus erythematosus (SLE) or with other autoimmune disorders as secondary APS, or can exist in isolation as primary antiphospholipid syndrome (pAPS). APS manifests with microvascular thrombosis as well as large-vessel occlusion with acute thrombotic episodes, which may involve all organ systems. Involvement of the central nervous

system (CNS) is common in patients with pAPS (Table 21.1). The high frequency of neurologic involvement in APS might be due to vulnerability of the cerebral vasculature, where the thrombotic event is more likely to be symptomatic than in the periphery.5 Neurologic complications such as transient ischemic attack and ischemic stroke have been described as typical features since the first report.1