原发性抗磷脂抗体综合征伴间歇性脑静脉窦血栓形成伴复发性癫痫1例

Tanwar Vs, Singh Aa, T. R., S. A, Jatana S, S. S
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摘要

抗磷脂综合征(APS)是一种获得性自身免疫性临床疾病,其特征是抗磷脂抗体水平持续升高,临床表现为高凝状态(复发性血管血栓形成和妊娠疾病)。原发性APS本质上是特发性的,尽管继发性APS与SLE等其他结缔组织疾病有关。在此,我们报告了一例原发性APS伴复发性脑静脉窦血栓形成的年轻未婚女性,其临床表现为全身性癫痫。抗心磷脂(aCL)和抗β2 GP1抗体的水平在相隔12周的两次升高。患者接受了抗凝治疗和抗癫痫治疗,治疗效果良好。该病例报告强调,临床医生应始终怀疑有癫痫发作史和脑成像中缺血性或血栓性病变的女性患者患有APS,无论是否存在妊娠合并症。关键词:抗磷脂综合征;脑静脉;窦血栓形成;癫痫
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A case of primary antiphospholipid antibody syndrome with intermittent cerebral venous sinus thrombosis manifested with recurrent epilepsy
Antiphospholipid syndrome (APS) is an acquired autoimmune clinical disorder characterized by persistently raised levels of anti-phospholipid antibodies and clinical feature of hypercoagulability (recurrent vascular thrombosis and pregnancy morbidities). Primary APS is idiopathic in nature though secondary APS is associated with other connective tissue diseases like SLE. Here, we present an instance of primary APS with recurrent cerebral venous sinus thrombosis in a youthful unmarried female who clinically presented with generalized epilepsy. Level of anti-cardiolipin (aCL) and anti-beta 2 GP1 antibodies were found elevated on two occasions 12 weeks apart. The patient was treated with anticoagulation therapy along with anti-epileptic therapy and she responded well to the therapy. This case report highlights that clinicians should always suspect APS in female patients who had history of seizures and ischemic or thrombotic lesions on brain imaging, irrespective of presence of pregnancy comorbidities. Keywords: antiphospholipid syndrome; cerebral venous; sinus thrombosis; epilepsy
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