Neurologic manifestations of antiphospholipid syndrome

Abstract

Neurological involvement in antiphospholipid antibody syndrome (APS) is common, and its occurrence increases morbidity and mortality. Patients may present variable neurological involvement, such as cerebrovascular disease, cognitive dysfunction, headache, seizures, movement disorders, multiple sclerosis-like syndrome, transverse myelitis and ocular symptoms. Most neurological manifestations are associated with thrombosis of the microcirculation or of large vessels; nonetheless, there is compelling evidence suggesting that, in some cases, symptoms are secondary to an immune-mediated pathogenesis, with direct binding of aPL on neurons and glia. Herein we describe clinical characteristics and management of neurological APS manifestations.

The burden of headache following aneurysmal subarachnoid hemorrhage: a prospective single-center cross-sectional analysis

“…Interestingly, all three SAH patients with anamnestic prior rheumatic condition reported on burdensome headache at follow-up, but this association narrowly failed the statistical significance. Being described already in 1938 by Cyriax, many recent reports chronicle headache in conjunction with various rheumatic conditions like polymyalgia rheumatica, systemic lupus erythematosus, and antiphospholipid syndrome [11,14,53,64]. Conversely, people with headache had a higher prevalence of rheumatic disorders in an Austrian general population-based study [55].…”

Mononeuritis Multiplex Associated With Antiphospholipid Antibodies

“…cognitive dysfunction, migraine, myelitis, seizure, chorea, leukoencephalopathy, Guillain-Barré syndrome, multiple sclerosis like syndrome). 4,5 Besides ischemic stroke, myelitis and some of the non-thrombotic features, other neurologic manifestations are rare, mainly thus related to the peripheral nervous system; nevertheless, they may involve a greater morbidity if diagnosis and treatment are not performed in a timely manner. Mononeuritis multiplex development has been rarely associated with antiphospholipid antibodies; as far as we know, there are only three cases reported.…”

Antiphospholipid Syndrome and the Neurologist: From Pathogenesis to Therapy

“…In a cohort of 55 APS patients, 25 of which suffered an ischemic stroke, the most common site of occlusion was the middle cerebral artery (31%) ( 50 ). In situ thrombosis is thought to be the most frequent pathogenic mechanism, whereas in other cases, cardiac embolisms can arise from involvement of the cardiac valvular apparatus, with thickening of valve leaflets by deposition of immune complexes (Liebman-Sacks endocarditis) ( 51 ). Besides stem or branch occlusion of intracranial arteries, a vasculitis-like pattern, with multiple sites of narrowing and dilation has also been described through arteriography ( 52 ).…”

“…Presence of aPL in non-SLE patients with CVT has been reported in previous studies ( 56 ). aCL positivity may be found in 7–22% of patients ( 51 ). APS accounts for ~6–17% of all CVT cases, being one of the most frequent prothrombotic conditions associated ( 57 ).…”

“…The pathogenesis of movement disorders in the setting of APS is matter of debate. On the one hand, cerebral infarctions and white matter changes on MRI suggest a thrombo-occlusive mechanism, which could account for the majority of cases ( 51 , 75 ). On the other, immune-mediated attack against basal ganglia epitopes has been suggested and reported in some cases ( 41 ).…”