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.

Keywords:
Lupus anticoagulant, Antiphospholipid antibodies, Dementia, Primary antiphospholipid syndrome
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© 1992 S. Karger AG, Basel
1992
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