APS and the Nervous System

Abstract

Cerebrovascular disease is the most frequent clinical manifestation of the antiphospholipid syndrome (APS) at disease onset, after deep venous thrombosis. At a 10-year follow-up, it represents the overall most common clinical event related to antiphospholipid antibodies (aPL). Besides thrombotic events affecting brain circulation, a wide range of “non-criteria” neurological manifestations has been associated with aPL such as dementia, epilepsy, chorea, headache, multiple sclerosis, myelopathy, peripheral neuropathy, hearing loss, and ocular syndromes. aPL display a particular tropism for cerebral circulation, which might be partially explained by the peculiarity of brain endothelial cells. However, aPL might induce neurological manifestations not only because of pro-thrombotic mechanisms but also because they can bind to neurons and astrocytes, disrupting their function. Ischemic manifestations of APS always require the initiation of either antiplatelet drugs or long-term anticoagulants; no standard treatment is available for nonvascular neurological manifestations of APS. The wide heterogeneity in neurological presentation of APS represents a challenge for clinicians: it is important to promptly recognize and effectively treat them in early stages, in order to avoid diagnostic and therapeutic delay.