Antiphospholipid Syndrome and the Neurologist: From Pathogenesis to Therapy

Abstract

Antiphospholipid syndrome (APS) is an autoimmune antibody-mediated condition characterized by thrombotic events and/or pregnancy morbidity in association with persistent positivity to antiphospholipid antibodies (aPL). The nervous system is frequently affected, as intracranial vessels are the most frequent site of arterial pathology. Over the course of years, many other neurological conditions not included in the diagnostic criteria, have been associated with APS. The pathogenic mechanisms behind the syndrome are complex and not fully elucidated. aPL enhance thrombosis, interfering with different pathways. Nevertheless, ischemic injury is not always sufficient to explain clinical features of the syndrome and immune-mediated damage has been advocated. This may be particularly relevant in the context of neurological complications. The reason why only a subgroup of patients develop non-criteria nervous system disorders and what determines the clinical phenotype are questions that remain open. The double nature, thrombotic and immunologic, of APS is also reflected by therapeutic strategies. In this review we summarize known neurological manifestations of APS, revisiting pathogenesis and current treatment options.

Clinical Characteristics and Management of Cerebral Venous Sinus Thrombosis in Patients With Antiphospholipid Syndrome: A Single-Center Retrospective Study

“…The involvement of aPLs in clinically significant normal procoagulant and anticoagulant reactions and on certain cells altering the expression and secretion of various molecules are the basis for possible mechanisms. Previous studies indicated that aPLs could induce a prothrombotic state through: (a) potentiating platelet aggregation and adhesiveness through upregulation of the thromboxane A2 23 ; (b) enhancing surface expression of tissue factor expression on endothelial cells and activate the extrinsic coagulation pathway 27 ; and (c) activation of the classical complement pathway and interference with endogenous anticoagulant mechanisms. 28 To date, the conclusion about the triple positivity (LACpositive, aCL-positive, and anti-b2GP-1 positive) have the highest risk for venous thrombosis 29 are inconsistent in previous studies.…”

<p>Enlarging acute tentorial subdural hematoma evacuated by surgery</p>

“…APS is an autoimmune antibody-mediated disorder marked by thrombotic events 10,11. To date, antithrombotic therapy is key in its management,10, and warfarin is regarded as the standard preventative treatment 11.…”

Immune Axonal Neuropathies Associated With Systemic Autoimmune Rheumatic Diseases

“…Additionally, a direct autoantibody-induced aggression is supposedly pathogenic (Mizisin and Weerasuriya, 2011). Antiphospholipid antibodies are linked to severe neural lesions (Snoussi et al, 2019) and are thought to induce ischemic damage by thrombosis of vasa nervorum (Imam et al, 2019) or vasculitis (Santos et al, 2010;Fleetwood et al, 2018). Antigenic determinants of the myelin phospholipids might be targets for anticardiolipin antibodies, leading to demyelinating neuropathies, as suggested by Santos et al (Santos et al, 2010).…”