CNS dysfunction in the antiphospholipid syndrome.
Though many neurological deficits have been described in the antiphospholipid syndrome (APS), only stroke is well established and accepted as a diagnostic criterion in this disease. We review clinical data obtained from a large series of cases regarding stroke, dementia, epilepsy, chorea, migraine, white matter disease and behavioral changes in 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 beta2-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 potentially valuable as a system in which to study the pathogenesis and 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.
Katzav
A
A
Department of Neurology, Sheba Medical Center, Tel Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Israel.
Chapman
J
J
Shoenfeld
Y
Y
D000818:Animals; D017152:Antibodies, Antiphospholipid / Q000032:analysis / Q000276:immunology*; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000276:immunology; D015415:Biomarkers / Q000032:analysis; D002493:Central Nervous System Diseases / Q000150:complications* / Q000175:diagnosis* / Q000276:immunology; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D011379:Prognosis; D018570:Risk Assessment; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index
D017152:Antibodies, Antiphospholipid; D015415:Biomarkers