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伴有严重蛋白S缺乏的罕见病例中脑静脉窦血栓形成的早期再通

Early recanalisation of cerebral venous sinus thrombosis in an unusual case associated with severe protein S deficiency.

作者信息

Verma Rajesh, Patil Tushar B, Kumar Neeraj

机构信息

Department of Neurology, Chhatrapati Shahuji Maharaj Medical University, Lucknow, Uttar Pradesh, India.

出版信息

BMJ Case Rep. 2012 Jul 27;2012:bcr2012006496. doi: 10.1136/bcr-2012-006496.

Abstract

An inherited or acquired deficiency of protein S leads to a prothrombotic state, with predisposition to venous thrombosis. We describe a case of cerebral venous sinus thrombosis (CVST) associated with acquired protein S deficiency and recanalisation within 15 days of anticoagulation. A 38-year-old man presented with recurrent headache, vomiting, altered sensorium and one episode of transient left hemiparesis. Magnetic resonance venography showed poor flow in the deep cerebral venous sinuses with extensive collateral venous channel formation, which resolved after 15 days of anticoagulation, along with clinical improvement. Serum protein S activity was found to be markedly low (16% of biological reference). CVST should be suspected in a patient with acute features of raised intracranial pressure or focal neurological deficit, and a patient without obvious clinical predisposition for a prothrombotic state should be evaluated for underlying thrombophilic states like protein S deficiency.

摘要

遗传性或获得性蛋白S缺乏会导致血栓形成倾向,易引发静脉血栓。我们描述了一例与获得性蛋白S缺乏相关的脑静脉窦血栓形成(CVST)病例,该患者在抗凝治疗15天内实现了再通。一名38岁男性出现反复头痛、呕吐、意识改变以及一次短暂性左半身轻瘫发作。磁共振静脉血管造影显示大脑深部静脉窦血流不佳,伴有广泛的侧支静脉通道形成,抗凝治疗15天后这些情况得到缓解,同时临床症状也有所改善。血清蛋白S活性显著降低(为生物学参考值的16%)。对于有颅内压升高或局灶性神经功能缺损急性症状的患者,应怀疑患有CVST,对于无明显血栓形成倾向临床易患因素的患者,应评估是否存在蛋白S缺乏等潜在的易栓症。

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本文引用的文献

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Recanalisation of cerebral venous thrombosis.脑静脉血栓形成的再通
J Neurol Neurosurg Psychiatry. 2003 Apr;74(4):459-61. doi: 10.1136/jnnp.74.4.459.
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