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神经白塞病一例,表现为腰椎脊髓受累。

A case of neuro-Behcet's disease presenting with lumbar spinal cord involvement.

机构信息

Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China.

出版信息

Spinal Cord. 2010 Feb;48(2):172-3. doi: 10.1038/sc.2009.124. Epub 2009 Sep 29.

Abstract

STUDY DESIGN

Case report.

OBJECTIVES

To report an unusual case of neuro-Behcet's disease (NBD) presenting with isolated myelitis that affected the lumbar cord.

SETTING

Department of Neurology, West China Hospital, Chengdu, Sichuan, China.

METHODS

A 19-year-old man presented with a 3-day history of paresthesia and weakness in both the legs. He had a 6-year history of recurrent oral and genital ulcers with erythema nodosum on his legs. Magnetic resonance imaging (MRI) revealed a T1-hypointense and T2-hyperintense lesion at the L1 level. Nodular enhancement of the lesion was achieved with gadolinium administration. Biopsy specimens from cutaneous lesions were consistent with an active perivasculitic process.

RESULTS

Corticosteroid (pulsed methylprednisolone followed by oral prednisolone) therapy was administered immediately. Subsequently, the patient's clinical condition improved dramatically. The spinal cord lesion disappeared on the MRI performed 1 year later. Thus far, there has been no recurrence.

CONCLUSION

NBD may involve the lumbar spinal cord. Prompt recognition of this disease and early vigorous steroid therapy are important to prevent further disability and recurrence.

摘要

研究设计

病例报告。

目的

报告一例以孤立性脊髓炎为表现的神经白塞病(NBD)的罕见病例,该脊髓炎影响到腰椎脊髓。

地点

中国四川成都华西医院神经内科。

方法

一名 19 岁男性因双下肢感觉异常和无力而就诊,病史 3 天。他有 6 年的复发性口腔和生殖器溃疡病史,腿部有红斑结节。磁共振成像(MRI)显示 L1 水平有 T1 低信号和 T2 高信号病变。病变部位行钆增强后呈结节状强化。皮肤病变的活检标本与活动性血管周围炎症过程一致。

结果

立即给予皮质类固醇(脉冲甲基强的松龙序贯口服泼尼松龙)治疗。随后,患者的临床状况显著改善。1 年后行 MRI 显示脊髓病变消失。迄今为止,无复发。

结论

NBD 可能累及腰椎脊髓。及时认识到这种疾病,并尽早进行强有力的类固醇治疗,对于防止进一步残疾和复发非常重要。

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