[Neurological Manifestations in Antiphospholipid Syndrome].

Abstract

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by persistent presence of positive antiphospholipid antibodies (aPLs). Historically, aPLs first attracted attention at the beginning of the 1970s, as a risk factor for the development of myelitis via cross-reaction with cephalin and sphingomyelin as neuronal tissue-enriched phospholipids. Primary APS manifestations include arteriovenous thrombosis and pregnancy complications; however, in rare cases, aPL-related neurological disorders including cognitive dysfunction, chorea, and transverse myelitis are observed. The pathogenesis and therapeutic strategies for thrombosis, including cerebral infarction in APS, have been established from basic and clinical research to date. However, pathological insights and clinical perspectives have not yet been well-defined for aPL-related cognitive dysfunction, chorea, and myelitis. Based on past experiences and findings of small observational studies, some patients with aPL-related neurological disorders recover following antiplatelet and anticoagulation therapy, but in some cases, multidisciplinary treatments with glucocorticoids and/or immunosuppressive agents, including cyclophosphamide, azathioprine, mycophenolate and rituximab, plasmapheresis, and psychoeducational support are required. A detailed research on pathophysiology and nationwide or international multicenter clinical trials for therapeutic strategies are vital for establishing sufficient basic and clinical understanding of aPL-related neurological manifestations.