作为抗磷脂综合征起病症状的神经学表现:附两例报告

Saeed Shahbeigi, S. Karamolahi, H. Pakdaman, S. Nazarbaghi, A. Altıntaş, Ommega Internationals
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引用次数: 1

摘要

抗磷脂综合征(APS)的定义是出现多种静脉和动脉血栓形成。在这种情况下,抗磷脂抗体(aPL),即狼疮抗凝血剂(LA),抗心磷脂抗体(acl)或抗β 2糖蛋白1抗体的存在是诊断的必要条件[1,2]。在一般人群中,aPL血清阳性的患病率在1-5%之间,但只有少数人会发展为APS[1]。为了明确诊断APS,必须观察到至少一项临床特征,如血管血栓形成或妊娠发病率和一项实验室异常。实验室异常必须至少间隔12周出现两次或两次以上[3]。APS累及大脑较为常见,临床表现各异;它们可能是第一个表现特征,也可能出现在疾病的过程中。脑受累的报告表现为:脑缺血事件,如CVA和TIA,癫痫,痴呆,认知障碍,头痛,精神障碍,舞蹈病,ms样综合征,横贯脊髓炎和眼部症状。值得注意的是,在没有APS标准的患者中存在aPL也可能与神经精神和认知障碍有关。
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The Neurological Manifestations as an Onset Symptom of Antiphospholipid Syndrome: Report of Two Cases
The Antiphospholipid Syndrome (APS) is defined by the occurrence of multiple venous and arterial thrombosis. In this condition, the presence of anti-phospholipid antibodies (aPL), namely lupus anticoagulant (LA), anti-Cardiolipin antibodies (acl) or anti-beta 2 glycoprotein 1 antibodies is necessary for diagnosis[1,2]. The prevalence of aPL seropositive ranges between 1-5% in the general population, but only a minority of these individuals develop the APS[1]. For the definite diagnosis of APS at least one clinical feature such as vascular thrombosis or pregnancy morbidity and one laboratory abnormality must be observed. The laboratory abnormalities must be present on two or more occasions at least 12 weeks apart[3]. The cerebral involvement in APS is common and characterized by different clinical manifestations; they could be the first presenting feature or appear in the course of the disease. The reported manifestations of the cerebral involvement are: cerebral ischemic events such as CVA and TIA, epilepsy, dementia, cognitive deficit, headache, psychiatric disorders, chorea, MSlike syndrome, transverse myelitis and ocular symptoms. It is notable to mention that the presence of aPL in patients without criteria for APS may also be associated with neuropsychiatric and cognitive disturbances.
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