Sitzer M, Söhngen D, Siebler M, Specker C, Rademacher J, Janda I, Aulich A, Steinmetz H
Department of Neurology, Heinrich-Heine-University, Düsseldorf, Germany.
Arch Neurol. 1995 Mar;52(3):271-5. doi: 10.1001/archneur.1995.00540270063020.
The pathogenesis of Sneddon's syndrome is unclear. This study addresses the question whether cerebral thromboembolism may be involved in the pathogenesis of the neurologic complications of the disorder. The study consisted of 13 patients with Sneddon's syndrome defined by both generalized livedo reticularis and a history of one or more cerebrovascular ischemic events; none had clinical or Doppler ultrasonographic evidence of atherosclerosis.
Transcranial Doppler microembolic monitoring of the middle cerebral artery; blood screening for antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibodies).
Five patients (38%) showed clinically silent microembolism at transcranial Doppler monitoring, with individual microembolic event rates of the middle cerebral artery between 2 per hour and 33 per hour. In this group, the time since the last ischemic symptom was significantly shorter than in the eight patients without microemboli. Antiphospholipid antibodies were detected in three patients (23%), all of whom belonged to the microemboli-positive group.
These data suggest that the detectability of both clinically silent cerebral microembolism and antiphospholipid antibodies may provide paraclinical evidence of active disease in patients with Sneddon's syndrome. The results support the notion that an immune-mediated prothrombotic state facilitating the formation of arterial thrombi with subsequent cerebral embolization, and/or triggering in situ thrombosis of cerebral vessels, plays a pathogenetic role in the neurologic manifestations of this disorder.
Sneddon综合征的发病机制尚不清楚。本研究探讨脑血栓栓塞是否可能参与该疾病神经并发症的发病机制。该研究纳入了13例Sneddon综合征患者，其定义为全身性网状青斑以及有一次或多次脑血管缺血事件的病史；均无动脉粥样硬化的临床或多普勒超声证据。
经颅多普勒监测大脑中动脉微栓塞情况；进行抗磷脂抗体（狼疮抗凝物、抗心磷脂抗体）的血液筛查。
5例患者（38%）在经颅多普勒监测时显示临床无症状性微栓塞，大脑中动脉的个体微栓塞事件发生率为每小时2次至每小时33次。在该组中，自上次缺血症状出现以来的时间明显短于8例无微栓塞的患者。3例患者（23%）检测到抗磷脂抗体，所有这些患者均属于微栓塞阳性组。
这些数据表明，临床无症状性脑微栓塞和抗磷脂抗体的可检测性可能为Sneddon综合征患者的活动性疾病提供临床旁证。结果支持这样一种观点，即免疫介导的促血栓形成状态促进动脉血栓形成并随后发生脑栓塞，和/或触发脑血管原位血栓形成，在该疾病的神经表现中起致病作用。
