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一名儿童的神经白塞病

Neuro-Behçets in a Child.

作者信息

Vesoulis Zachary A, Galindo Rafael, Ornstein Bradley W, White Andrew J

机构信息

Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA.

Department of Neurology, Division of Pediatric & Developmental Neurology, Washington University School of Medicine, St. Louis, MO, USA.

出版信息

Child Neurol Open. 2014 Nov 10;1(1):2329048X14550505. doi: 10.1177/2329048X14550505. eCollection 2014 Jul-Sep.

DOI:10.1177/2329048X14550505
PMID:28503582
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5417034/
Abstract

We describe a case of neuro-Behçet disease diagnosed in a 12-year-old girl. This patient presented with recurrent oral ulcers, incontinence, spastic gait, blurry vision, and asymmetrical lower extremity hypertonia. Extensive testing revealed punctate lesions through the central nervous system, vitritis, papillitis, and uveitis. A thorough infectious and neoplastic workup was negative. She was treated with pulse steroids and azathioprine with gradual improvement in her gait and ophthalmologic findings. Although rare, primary neuro-Behçet should be considered in pediatric patients with neurologic abnormalities and recurrent aphthous ulcers without other explanation.

摘要

我们描述了一例在一名12岁女孩中诊断出的神经白塞病病例。该患者出现复发性口腔溃疡、尿失禁、痉挛性步态、视力模糊和双下肢不对称性张力亢进。广泛检查发现贯穿中枢神经系统的点状病变病变、玻璃体炎、视乳头炎和葡萄膜炎。全面的感染性和肿瘤性检查结果均为阴性。她接受了脉冲类固醇和硫唑嘌呤治疗,步态和眼科检查结果逐渐改善。虽然罕见,但对于有神经异常和复发性阿弗他溃疡且无其他解释的儿科患者,应考虑原发性神经白塞病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6fc6/5417034/96ed79e48e9a/10.1177_2329048X14550505-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6fc6/5417034/b649d66a74f3/10.1177_2329048X14550505-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6fc6/5417034/65d674b08248/10.1177_2329048X14550505-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6fc6/5417034/96ed79e48e9a/10.1177_2329048X14550505-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6fc6/5417034/b649d66a74f3/10.1177_2329048X14550505-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6fc6/5417034/65d674b08248/10.1177_2329048X14550505-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6fc6/5417034/96ed79e48e9a/10.1177_2329048X14550505-fig3.jpg

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本文引用的文献

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Neurological involvement in paediatric Behçet's disease.小儿白塞病的神经系统受累情况。
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Anti-TNF agents for Behçet's disease: analysis of published data on 369 patients.用于治疗白塞病的 TNF 拮抗剂:对 369 名患者发表数据的分析。
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EULAR recommendations for the management of Behçet disease.欧洲抗风湿病联盟(EULAR)关于白塞病管理的建议。
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MEFV mutations are increased in Behçet's disease (BD) and are associated with vascular involvement.在白塞病(BD)中,MEFV突变增加,且与血管受累相关。
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