Sakamoto Shingo, Akutsu Koichi, Kawase Kayoko, Takada Tatsuro, Seyama Hidenori, Takahashi Jun, Miyamoto Susumu, Nonogi Hiroshi, Takeshita Satoshi
Department of Medicine, National Cardiovascular Center, Osaka, Japan.
Angiology. 2008;59(6):765-8. doi: 10.1177/0003319707309310. Epub 2008 Apr 2.
Antiphospholipid syndrome is characterized with arterial and venous thrombosis. In this article, a 38-year-old man presented with headache and swelling of his left limb, which had lasted for 2 months. Duplex ultrasonography showed thrombosis of the left femoral vein. Cerebral magnetic resonance imaging also showed cerebral sinus thrombosis. Serological examination showed that antiphospholipid syndrome was the underlying disease condition in this patient. Despite adequate anticoagulant therapy, deep vein thrombosis and cerebral sinus thrombosis exacerbated, resulting in inferior vena cava occlusion, papilloedema, and abducent nerve paralysis. Optic canal decompression and cistern-peritoneal shunt operation were performed, following which his neurological symptoms were relieved. The occurrence of cerebral sinus thrombosis as initial presentation of antiphospholipid syndrome is extremely rare and remains a diagnostic challenge. Although the clinical presentation is highly variable, the diagnosis should be considered in patients with antiphospholipid syndrome presenting with recent unusual headaches. Improved diagnosis and treatment strategy may ultimately improve the clinical outcome of these patients.
抗磷脂综合征的特征是动脉和静脉血栓形成。在本文中,一名38岁男性出现头痛和左下肢肿胀,持续了2个月。双功超声检查显示左股静脉血栓形成。脑磁共振成像也显示脑窦血栓形成。血清学检查表明抗磷脂综合征是该患者的潜在疾病状况。尽管进行了充分的抗凝治疗,但深静脉血栓形成和脑窦血栓形成仍加剧,导致下腔静脉闭塞、视乳头水肿和展神经麻痹。进行了视神经管减压和脑池-腹腔分流手术,术后其神经症状得到缓解。脑窦血栓形成作为抗磷脂综合征的首发表现极为罕见,仍然是一个诊断挑战。尽管临床表现高度多变,但对于近期出现异常头痛的抗磷脂综合征患者应考虑进行诊断。改进的诊断和治疗策略最终可能改善这些患者的临床结局。