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以延髓病变为表现的神经白塞病:一例报告

Neuro-Behçet Disease Presenting as a Bulbar Lesion: A Case Report.

作者信息

Certal Mariana, Alves Ana Sofia, Santos Marta B, Salvador Fernando, Mendes Michel

机构信息

Internal Medicine, Unidade Local de Saúde (ULS) de Trás-os-Montes e Alto Douro, Chaves, PRT.

Internal Medicine, Unidade Local de Saúde (ULS) de Trás-os-Montes e Alto Douro, Vila Real, PRT.

出版信息

Cureus. 2024 Aug 30;16(8):e68236. doi: 10.7759/cureus.68236. eCollection 2024 Aug.

DOI:10.7759/cureus.68236
PMID:39347354
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11439451/
Abstract

Behçet's disease (BD), also called neuro-Behçet (NB), is a multisystem inflammatory disease that can affect the nervous system. The authors hereby present the case of a 46-year-old woman with a previous diagnosis of BD with cutaneous, articular, and ocular involvement. The patient was admitted to the emergency room with intense posterior cervical pain, right eyelid ptosis accompanied by anisocoria, left arm hemiparesis, left hemihypoesthesia, right dysmetria, and postural instability. A cerebral MRI revealed a right laterobulbar oval T2 hyperintense lesion, suggesting a diagnosis of NB. Treatment with methylprednisolone pulses and, later, azathioprine was then started. The patient showed progressive improvement in her clinical condition and an imagological resolution of the bulbar lesion. This case highlights the importance of an accurate diagnosis - based on clinical history and imaging studies - for an early initiation of the specific therapy.

摘要

白塞病(BD),也称为神经白塞病(NB),是一种可累及神经系统的多系统炎症性疾病。作者在此介绍一例46岁女性病例,该患者先前被诊断为患有皮肤、关节和眼部受累的白塞病。患者因剧烈的颈后部疼痛、右眼睑下垂伴瞳孔不等大、左臂偏瘫、左半身感觉减退、右辨距不良和姿势不稳而被送入急诊室。脑部磁共振成像(MRI)显示右侧延髓外侧椭圆形T2高信号病变,提示诊断为神经白塞病。随后开始用甲泼尼龙冲击治疗,之后使用硫唑嘌呤。患者的临床状况逐渐改善,延髓病变在影像学上得到消退。该病例强调了基于临床病史和影像学研究进行准确诊断对于早期开始特异性治疗的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/117a/11439451/967d20c00afa/cureus-0016-00000068236-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/117a/11439451/967d20c00afa/cureus-0016-00000068236-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/117a/11439451/967d20c00afa/cureus-0016-00000068236-i01.jpg

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本文引用的文献

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Update on the Diagnosis of Behçet's Disease.白塞病诊断的最新进展
Diagnostics (Basel). 2022 Dec 23;13(1):41. doi: 10.3390/diagnostics13010041.
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Neuro-Behcet's disease: An update on diagnosis, differential diagnoses, and treatment.神经白塞病:诊断、鉴别诊断及治疗的最新进展
Mult Scler Relat Disord. 2020 Apr;39:101906. doi: 10.1016/j.msard.2019.101906. Epub 2019 Dec 23.
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Behçet's Disease: An Overview of Etiopathogenesis.白塞病:发病机制概述。
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The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center.白塞病的长期死亡率和发病率:在一个专门中心对387例患者进行的20年随访结果调查
Medicine (Baltimore). 2003 Jan;82(1):60-76. doi: 10.1097/00005792-200301000-00006.
8
Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group.白塞病神经受累的临床模式:200例患者的评估。神经白塞病研究组
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