脑静脉窦血栓形成作为首发的神经白塞病1例报告并文献复习

doi:10.3969/j.issn.1004-583X.2021.02.014

摘要/Abstract

摘要：

神经白塞病(Neuro-Behcet's disease, NBD)指的是白塞病(Behcet's disease, BD)患者神经系统受累。NBD中枢神经系统表现分为实质型和非实质型,脑静脉窦血栓形成(cerebral venous sinus thrombosis, CVST)为NBD非实质型中主要表现。CVST是BD患者的少见表现,通常有颅高压表现,如头痛、呕吐、视乳头水肿等。由于BD无特异性诊断检查,主要依靠临床诊断,当年轻男性患者以持续性头痛,颅内压增高等表现时,需考虑是否有BD合并CVST的发生。在此我们介绍一名年轻男性患者以CVST为首发表现的NBD并对BD合并CVST相关文献进行回顾与学习。

关键词: 窦血栓形成, 颅内, 贝赫切特综合征

Abstract:

Neuro-Behcet's disease (NBD) represents the involvement of the nervous system in patients with Behcet's disease (BD). The central nervous system manifestations of NBD are divided into parenchyma type and non-parenchyma type. Cerebral venous sinus thrombosis(CVST) serves as main manifestation of NBD non-parenchymal type. CVST, a rare manifestation in BD patients, is usually complicated with cranial hypertension involving the headache, vomitin, and papilledema. BD mainly relies on clinical diagnosis due to the lack of specific diagnostic test. It is necessary to consider the occurrence of BD with CVST for young male patients suffering from persistent headache and increased intracranial pressure. A young male patient with CVST as the first manifestation is introduced, and the literature related to BD combined with CVST are reviewed.

Key words: sinus thrombosis, intracranial, Behcet syndrome

中图分类号:

R743.33

尹园园, 任慧玲. 脑静脉窦血栓形成作为首发的神经白塞病1例报告并文献复习[J]. 临床荟萃, 2021, 36(2): 162-167.

Yin Yuanyuan, Ren Huiling. Cerebral venous sinus thrombosis as the first manifestation of Neuro-Behcet's disease: one case report and literature review[J]. Clinical Focus, 2021, 36(2): 162-167.

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链接本文: https://huicui.hebmu.edu.cn/CN/10.3969/j.issn.1004-583X.2021.02.014

https://huicui.hebmu.edu.cn/CN/Y2021/V36/I2/162

图/表 5

图1 OCT检查 a.治疗前一侧视乳头水肿;b.治疗后视乳头边界清楚,水肿减轻

图2 白塞病的其他症状及体征 a.口腔有一3 mm×3 mm大小溃疡;b.阴囊处有散在溃疡瘢痕;c.下肢可见浅静脉炎;d.针刺试验阳性

图3 颅脑CT与DWI均提示上矢状窦血栓形成(红色箭头)

图4 颅脑MRI平扫及增强 a.MRI T1相;b.MRI T2相;c.MRI FLAIR相,上矢状窦处均出现空三角征(红色箭头),提示静脉窦血栓的形成;d~f.为增强核磁,显示上矢状窦中心出现三角形信号减低区,周围包绕高信号环(红色箭头)

图5 颅脑MRV a~b.示治疗前上矢状窦后部显影不均匀(红色箭头),右侧横窦显影不佳,变细,右侧乙状窦及颈内静脉未见明显显影(黄色圆圈内),考虑颅内静脉窦形成。c~d.示治疗后上矢状窦与右侧横窦、乙状窦及颈内静脉比之前有改善

参考文献 29

[1]	Borhani-Haghighi A, Kardeh B, Banerjee S, et al. Neuro-Behcet's disease: An update on diagnosis, differential diagnoses, and treatment[J]. Mult Scler Relat Disord, 2019,39:101906. doi: S2211-0348(19)30977-0 pmid: 31887565
[2]	Sakane T, Takeno M, Suzuki N, et al. Behçet's disease[J]. N Engl J Med, 1999,341(17):1284-1291. doi: 10.1056/NEJM199910213411707 URL
[3]	杨庆林, 王佳伟, 赖春涛. 白塞病相关卒中[J]. 中国卒中杂志, 2017,12(5):432-435.
[4]	Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group[J]. Brain, 1999,122(Pt 11):2171-2182. doi: 10.1093/brain/122.11.2171 URL
[5]	Al-Araji A, Kidd DP. Neuro-Behçet's disease: epidemiology, clinical characteristics, and management[J]. Lancet Neurology, 2009,8(2):192-204. doi: 10.1016/S1474-4422(09)70015-8 pmid: 19161910
[6]	Shi J, Huang X, Li G, et al. Cerebral venous sinus thrombosis in Behcet's disease: a retrospective case-control study[J]. Clin Rheumatol, 2018,37(1):51-57. doi: 10.1007/s10067-017-3718-2 URL
[7]	Saip S, Akman-Demir G, Siva A. Neuro-Behçet syndrome[J]. Handb Clin Neurol, 2014,121:1703-1723.
[8]	Ferro JM, Aguiar de Sousa D. Cerebral Venous Thrombosis: an Update[J]. Curr Neurol Neurosci Rep, 2019,19(10):74-74. doi: 10.1007/s11910-019-0988-x pmid: 31440838
[9]	Uluduz D, Midi I, Duman T, et al. Behcet's disease as a causative factor of cerebral venous sinus thrombosis: subgroup analysis of data from the VENOST study[J]. Rheumatology (Oxford), 2019,58(4):600-608. doi: 10.1093/rheumatology/key153 pmid: 29992235
[10]	de Sousa DA, Mestre T, Ferro JM. Cerebral venous thrombosis in Behçet's disease: a systematic review[J]. J Neurol, 2011,258(5):719-727. doi: 10.1007/s00415-010-5885-9 URL
[11]	Akdal G, Yaman A, Men S, et al. Pseudotumor cerebri syndrome without cerebral venous sinus thrombosis in Behcet's disease[J]. J Neurol Sci, 2017,383:99-100. doi: 10.1016/j.jns.2017.10.038 URL
[12]	Greco A, De Virgilio A, Ralli M, et al. Behçet's disease: New insights into pathophysiology, clinical features and treatment options[J]. Autoimmun Rev, 2018,17(6):567-575. doi: 10.1016/j.autrev.2017.12.006 URL
[13]	The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria[J]. J Eur Acad Dermatol Venereol, 2014,28(3):338-347. doi: 10.1111/jdv.12107 URL
[14]	Kirino Y, Ideguchi H, Takeno M, et al. Continuous evolution of clinical phenotype in 578 Japanese patients with Behçet's disease: a retrospective observational study[J]. Arthritis Res Ther, 2016,18(1):217. doi: 10.1186/s13075-016-1115-x URL
[15]	Takeuchi M, Mizuki N, Meguro A, et al. Dense genotyping of immune-related loci implicates host responses to microbial exposure in Behçet's disease susceptibility[J]. Nat Genet, 2017,49(3):438-443. doi: 10.1038/ng.3786 URL
[16]	Mizuki Y, Horita N, Horie Y, et al. The influence of HLA-B51 on clinical manifestations among Japanese patients with Behçet's disease: A nationwide survey[J]. Mod Rheumatol, 2019, 10.1080/14397595.2019.1649103:1-7.
[17]	Chamorro AJ, Marcos M, Hernández-García I, et al. Association of allelic variants of factor V Leiden, prothrombin and methylenetetrahydrofolate reductase with thrombosis or ocular involvement in Behçet's disease: a systematic review and meta-analysis[J]. Autoimmun Rev, 2013,12(5):607-616. doi: 10.1016/j.autrev.2012.11.001 URL
[18]	Krumb E, Lefebvre C, Peeters A, et al. Cerebral venous thrombosis revealing Behcet's disease in a Moroccan patient: A case report and literature review[J]. SAGE Open Med Case Rep, 2018, 6:2050313x18767053.
[19]	Brenière C, Blanc C, Devilliers H, et al. Associated arterial and venous cerebral manifestations in Behçet's disease[J]. Rev Neurol (Paris), 2018,174(5):337-341. doi: 10.1016/j.neurol.2017.06.031 URL
[20]	Yesilot N, Bahar S, Yilmazer S, et al. Cerebral venous thrombosis in Behçet's disease compared to those associated with other etiologies[J]. J Neurol, 2009,256(7):1134-1142. doi: 10.1007/s00415-009-5088-4 URL
[21]	Gao Y, Lyu S, Bao L, et al. Sudden unilateral vision loss in an adult with Behcet disease: A case report[J]. Medicine (Baltimore), 2018,97(50):e13594. doi: 10.1097/MD.0000000000013594 URL
[22]	Ksiaa I, Abroug N, Kechida M, et al. Eye and Behcet's disease[J]. J Fr Ophtalmol, 2019,42(4):e133-e146. doi: 10.1016/j.jfo.2019.02.002 pmid: WOS:000464334600001
[23]	Alghamdi A, Bodaghi B, Comarmond C, et al. Neuro-ophthalmological manifestations of Behçet's disease[J]. Br J Ophthalmol, 2019,103(1):83-87. doi: 10.1136/bjophthalmol-2017-311334 pmid: 29699980
[24]	Jalli R, Zarei F, Farahangiz S, et al. The sensitivity, specificity, and accuracy of contrast-enhanced t1-weighted image, t2*-weighted image, and magnetic resonance venography in diagnosis of cerebral venous sinus thrombosis[J]. J Stroke Cerebrovasc Dis, 2016,25(8):2083-2086. doi: 10.1016/j.jstrokecerebrovasdis.2016.01.039 pmid: 27177920
[25]	Kovacs MJ. Letter by Kovacs regarding article, “Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association”[J]. Stroke, 2011,42(7):e408.
[26]	Koennecke HC. Cerebral venous thrombosis in adults[J]. Vasa, 2019,48(6):473-482. doi: 10.1024/0301-1526/a000788 URL
[27]	Roriz M, Crassard I, Lechtman S, et al. Can anticoagulation therapy in cerebral venous thrombosis associated with Behcet's disease be stopped without relapse?[J]. Rev Neurol (Paris), 2018,174(3):162-166. doi: 10.1016/j.neurol.2017.06.021 URL
[28]	Terashita S, Tanaka T, Taneichi H, et al. Mycophenolate mofetil and prednisolone for cerebral sinus venous thrombosis with Behcet's disease[J]. Pediatr Int, 2019,61(9):920-922. doi: 10.1111/ped.13943 pmid: 31512337
[29]	Duman T, Uluduz D, Midi I, et al. A Multicenter Study of 1144 Patients with Cerebral Venous Thrombosis: The VENOST Study[J]. J Stroke Cerebrovasc Dis, 2017,26(8):1848-1857. doi: 10.1016/j.jstrokecerebrovasdis.2017.04.020 URL