Katzav A, Chapman J, Shoenfeld Y
Department of Neurology, Sheba Medical Center, Tel Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Israel.
Lupus. 2003;12(12):903-7. doi: 10.1191/0961203303lu500oa.
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.
尽管抗磷脂综合征（APS）中已描述了许多神经功能缺损，但只有 stroke 在该疾病中是公认的诊断标准。我们回顾了从大量关于 APS 中的 stroke、痴呆、癫痫、舞蹈症、偏头痛、白质病及行为改变或与抗磷脂抗体（aPL）等实验室标准相关病例中获取的临床数据。强调了动物模型对我们理解 APS 这些表现的贡献，特别是对于我们已在小鼠中建立模型系统的认知和行为方面。这些模型利用用β2 - 糖蛋白 I（APS 中的一种核心自身抗原）免疫小鼠，其可诱导持续高水平的 aPL。这些小鼠在四到五个月后会出现多动行为以及学习和记忆缺陷，作为研究 APS 认知和行为方面发病机制及治疗的系统可能具有重要价值。我们开发的另一个模型，其中 APS 患者的 IgG 可诱导脑突触神经小体去极化，可作为 APS 中癫痫发病机制的模型。
