神经性贝赫切氏症表现为脑静脉血栓--两例病例报告及文献综述。

Journal of postgraduate medicine Pub Date : 2024-07-01 Epub Date: 2024-07-12 DOI:10.4103/jpgm.jpgm_597_23
M Gupta, V B Rao, S Ramakrishnan, G B Kulkarni
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摘要

摘要:脑静脉血栓(CVT)是一种罕见的中风,有多种风险因素。贝赫切特病(BD)是一种罕见的危险因素。在我们中心过去 10 年的约 3000 例病例中,有两例 BD 合并 CVT。在此,我们将报告他们的临床症状、病程和治疗方法。病例 1 是一名 18 岁的女孩,有反复皮肤损伤病史,因 CVT 而出现脑病综合征,需要减压治疗。尽管我们尽了最大努力,但她还是出现了并发症,最终因败血症去世。病例 2 是一名 22 岁的男性,患有颅内压升高综合征和复发性生殖器溃疡。他的检查结果显示患有视网膜血管炎、乳头水肿和双侧直肌外侧麻痹。两人的神经影像学检查均显示有CVT,人类白细胞抗原-B51阳性。病例 2 对抗凝剂和免疫调节有反应。在处理 CVT 时,识别风险因素至关重要,而有计划的评估(临床或检查)在识别需要特殊治疗的罕见病因方面发挥着重要作用。
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Neuro-Behçet's presentation as cerebral venous thrombosis - A report of two cases and review of the literature.

Abstract: Cerebral venous thrombosis (CVT) is a rare stroke with multiple risk factors. One rare risk factor is Behçet's disease (BD). Out of around 3000 cases at our center in the past 10 years, two cases of BD with CVT were seen. Herein, we report on their clinical symptoms, course, and management. Case 1 was a 18-year-old girl with a history of recurrent skin lesions presenting with encephalopathy syndrome due to CVT, requiring decompression. Despite our best efforts, she developed complications and expired due to sepsis. Case 2 was a 22-year-old male with raised intracranial pressure syndrome and a history of recurrent orogenital ulcers. His evaluation showed retinal vasculitis, papilledema, and bilateral lateral rectus palsy. Both had CVT on neuroimaging and had positivity for human leukocyte antigen-B51. Case 2 responded to the anticoagulation and immunomodulation. Risk factor identification is essential in managing CVT, and planned evaluation (clinical or investigations) plays an important role in identifying rare causes that need specific treatment.

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