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神经贝赫切特病表现为脑静脉血栓形成——两例病例报告及文献复习。

Neuro-Behçet's presentation as cerebral venous thrombosis - A report of two cases and review of the literature.

机构信息

Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.

出版信息

J Postgrad Med. 2024 Jul 1;70(3):162-165. doi: 10.4103/jpgm.jpgm_597_23. Epub 2024 Jul 12.

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.

摘要

脑静脉血栓形成(CVT)是一种罕见的中风,有多种危险因素。一种罕见的危险因素是贝赫切特病(BD)。在我们中心过去 10 年的大约 3000 例病例中,有 2 例 BD 合并 CVT。在此,我们报告他们的临床症状、病程和治疗情况。病例 1 是一名 18 岁女孩,有反复发作的皮肤病变史,因 CVT 出现脑病综合征,需要减压。尽管我们尽了最大努力,她还是因败血症而出现并发症并死亡。病例 2 是一名 22 岁男性,有颅内压升高综合征和反复发作的口腔生殖器溃疡史。他的评估显示视网膜血管炎、视盘水肿和双侧外直肌瘫痪。两人的神经影像学均显示 CVT,且人类白细胞抗原-B51 阳性。病例 2 对抗凝和免疫调节治疗有反应。管理 CVT 时,识别危险因素至关重要,计划评估(临床或调查)在识别需要特定治疗的罕见病因方面发挥着重要作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1c47/11458080/15ef3632417b/JPGM-70-162-g001.jpg

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