The Lupus Anticoagulant and Dementia in Non-SLE Patients

Abstract

The syndrome of circulating anticoagulants in patients without evidence of systemic lupus erythematosus (SLE) has lately become a focus of interest. The clinical features, neuroimaging findings and clinical course of 12 such patients, who presented with neurological symptoms, were analyzed. None of the patients fulfilled the American Rheumatism Association (ARA) criteria of SLE. During a follow-up period of up to 5 years, 9 patients (75%) deteriorated mentally. In 5, cerebrovascular lesions were observed clinically and/or neuroradiologically. Four patients showed slowly progressive mental decline without focal deficits on clinical examination or on CT and MR studies of the brain, except for a small cortical infarct in one case. All patients had radiological evidence of cortical atrophy. Thus, dementia may be a prominent feature among patients with the lupus anticoagulant. Possible underlying mechanisms, such as multi-infarct state, as well as an autoimmune process affecting specifically CNS structures, are discussed.

Dementia and Antiphospholipid Antibodies

“…This proportion was significant when compared to age-matched controls. We also found that, in agreement with our previous observation [8], those who had high levels of aPLAb were elderly patients with DAT rather than with vascular dementia. It is expected that patients with high levels of aPLAb will develop vascular dementia and indeed, such a relationship was observed in younger stroke patients [11].…”

“…These antibodies are usually measured by binding to phospholipids in an enzyme-linked immunosorbent assay (ELISA) [1,2]. In this syndrome, as in systemic lupus erythematosus, the high levels of antiphospholipid antibodies (aPLAb) may be associated with neurological manifestations such as headache, stroke [3], chorea [4], epilepsy [5], psychosis [6] and dementia [7,8].…”

“…A followup of relatively young patients with high levels of aPLAb found that over 50% of them developed dementia [8]. Furthermore, aPLAb have been found to be associated with impaired cognitive function [9].…”

Antiphospholipid Antibodies Bind ATP: A putative Mechanism for the Pathogenesis of Neuronal Dysfunction

“…High levels of circulating aPL IgG have been implicated in neurological diseases such as focal cerebral ischemia, myelopathy, Guillain-Barre syndrome, migraine, chorea, epilepsy and dementia (Lubbe and Walker 1983, Coull et al 1987, Levine et al 1987, Inzelberg and Korczyn 1989, Inzelberg et al 1992, Herranz et al 1994, Levine et al 1995, Cervera et al 1997. The pathogenesis of nervous system disease associated with aPL IgG is still under investigation.…”

Cognitive Dysfunction and Antiphospholipid Antibodies

“…In a multicenter study including 1,000 patients with APS, multi-infarct dementia was observed in 2.5% of patients, but no data were provided on subtle forms of cognitive dysfunction [15]. In other studies focusing on dementia and MRI, a significantly higher prevalence of dementia was observed [16,17].…”