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干燥综合征患者复发性纵向广泛横贯性脊髓炎

Recurrent longitudinally extensive transversal myelitis in a patient with Sjögren's syndrome.

作者信息

Sekarić Jelena, Zivanović Zeljko, Lucić-Prokin Aleksandra, Kokai-Zekić Timea, Petrović Kosta, Turkalj Ivan, Jovin Zita, Gvozdenović Slobodan

机构信息

Department of Neurology, Clinical Centre of Vojvodina, Novi Sad, Serbia.

Department of Radiology, Clinical Centre of Vojvodina, Novi Sad, Serbia.

出版信息

Vojnosanit Pregl. 2013 Nov;70(11):1056-8. doi: 10.2298/vsp1311056s.

DOI:10.2298/vsp1311056s
PMID:24397203
Abstract

INTRODUCTION

Longitudinally extensive transverse myelitis (LETM) is a transversal myelitis that extends through three or more vertebral segments in length.

CASE REPORT

A 52-year-old woman was hospitalized due to pain in the lumbar region, difficulty in walking, hypoesthesia of the anogenital area and urinary retention. In the past medical history, two years earlier, the patient had been diagnosed with transversal myelitis confirmed by MRI of the cervical spine and six months earlier, the patient was diagnosed with primary Sjögren's syndrome (SS). During the current hospitalization MRM of the spinal cord revealed extensive inflammatory lesions of almost the whole spinal cord. Lumbar puncture (LP) revealed mild pleocytosis and slightly increased protein level. Isoelectric focusing of cerebrospinal fluid (CSF) and serum proteins was normal. Visual evoked potentials were normal. Serological testing excluded acute viral infections. Corticosteroid therapy was applied with good therapeutic response. Control MRI revealed regression of pathological changes in the spinal cord.

CONCLUSION

A wide range of disorders can cause LETM, but usually the first line diagnosis is neuromyelitis optica (NMO). Based on the detection of NMO immunoglobulin G in the serum of affected patients, a variety of allied disorders were grouped under the name of NMO spectrum disorders, including recurrent myelitis associated with LETM and myelitis associated with autoimmune disorders such as SS. There have been only a few cases reported in the literature with recurrent LETM associated with non-organ specific autoimmune disorder.

摘要

引言

长节段横贯性脊髓炎(LETM)是一种横贯性脊髓炎,其长度延伸超过三个或更多椎体节段。

病例报告

一名52岁女性因腰部疼痛、行走困难、肛门生殖器区域感觉减退及尿潴留入院。既往史显示,两年前患者经颈椎MRI确诊为横贯性脊髓炎,六个月前被诊断为原发性干燥综合征(SS)。此次住院期间,脊髓磁共振成像(MRM)显示几乎整个脊髓存在广泛炎症性病变。腰椎穿刺(LP)显示轻度淋巴细胞增多及蛋白水平轻度升高。脑脊液(CSF)和血清蛋白的等电聚焦正常。视觉诱发电位正常。血清学检测排除了急性病毒感染。应用皮质类固醇治疗,疗效良好。对照MRI显示脊髓病变消退。

结论

多种疾病可导致LETM,但通常一线诊断为视神经脊髓炎(NMO)。基于在受累患者血清中检测到NMO免疫球蛋白G,多种相关疾病被归为NMO谱系疾病,包括与LETM相关的复发性脊髓炎以及与自身免疫性疾病(如SS)相关的脊髓炎。文献中仅有少数几例与非器官特异性自身免疫性疾病相关的复发性LETM病例报道。

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