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Abstract

Though many neurological deficits have been described in the antiphospholipid syndrome (APS), only stroke is well establishedand accepted as a diagnosticcriterion in this disease. We review clinical data obtainedfrom a large series of cases regardingstroke, dementia, epilepsy, chorea, migraine, white matter disease and behavioralchangesin APS or linked to laboratory criteria such as antiphospholipid antibodies (aPL). The contribution of animal models to our understanding of these manifestations of APS is stressed, especially regarding the cognitive and behavioral aspects for which we have established model systems in the mouse. These models utilize immunization of mice with b2-glycoprotein I, a central autoantigen in APS, which induces persistent high levels of aPL. These mice develop hyperactive behavior after a period of four to five months as well as deficits in learning and memory and are potentiallyvaluableas a system in which to study the pathogenesisand treatment of cognitive and behavioral aspects of APS. Another model we have developed, in which IgG from APS patients induce depolarization of brain synaptoneurosomes, may serve as a model for the pathogenesis of epilepsy in APS.

Keywords

animal models antiphospholipid antibodies antiphospholipid syndrome behavior chorea dementia epilepsy neurology

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CNS dysfunction in the antiphospholipid syndrome

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