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干燥性脊髓炎表现为半侧脊髓综合征。

Sjogren's myelitis presenting as hemicord syndrome.

机构信息

Nottingham University Hospitals NHS Trust, Nottingham, UK.

Nottingham University Hospitals NHS Trust, Nottingham, UK, and consultant neurologist, University of Nottingham School of Medicine, Nottingham, UK

出版信息

Clin Med (Lond). 2023 Jul;23(4):423-424. doi: 10.7861/clinmed.2023-0158.

Abstract

We present a case where a 63-year-old right-handed man who presented with a 6-month history of progressive asymmetrical sensorimotor symptoms in lower limbs. This was associated with concomitant rash on the lower limbs, and mild sicca symptoms. MRI spine showed focal T2 hyperintensity in the left hemicord at C3-4 level. Skin biopsy of the rash revealed urticarial vasculitis, and lip biopsy revealed lymphocytic sialadenitis. Initial anti-Ro antibody was negative, but subsequent Ro52 antibody testing returned positive. There was also matched serum and cerebrospinal fluid oligoclonal bands. He was subsequently diagnosed as Sjogren's myelitis and treated with intravenous methylprednisolone, then transitioned to a steroid sparing agent. This case highlights the difficulties in reaching a rheumatological diagnosis in the early stages with typical negative antibodies, and shows a rare neurological manifestation of a systemic rheumatological condition.

摘要

我们报告了一例 63 岁的右利手男性,其主要表现为进行性、不对称的下肢感觉运动症状,病程 6 个月。下肢伴有皮疹,并有轻微的干燥症状。颈椎 MRI 显示 C3-4 水平左侧半脊髓的局灶性 T2 高信号。皮疹的皮肤活检显示荨麻疹性血管炎,唇活检显示淋巴细胞性唾液腺炎。最初的抗 Ro 抗体阴性,但随后的 Ro52 抗体检测呈阳性。同时还有血清和脑脊液寡克隆带。因此,他被诊断为干燥综合征性脊髓炎,并接受了静脉甲基强的松龙治疗,随后转为类固醇保存剂治疗。该病例强调了在早期阶段具有典型阴性抗体时,风湿学诊断的困难,并显示了一种罕见的系统性风湿性疾病的神经表现。

相似文献

1
Sjogren's myelitis presenting as hemicord syndrome.干燥性脊髓炎表现为半侧脊髓综合征。
Clin Med (Lond). 2023 Jul;23(4):423-424. doi: 10.7861/clinmed.2023-0158.

本文引用的文献

1
Neurologic Manifestations of Primary Sjögren Syndrome.原发性干燥综合征的神经系统表现
Rheum Dis Clin North Am. 2017 Nov;43(4):519-529. doi: 10.1016/j.rdc.2017.06.002. Epub 2017 Aug 23.
6
Clinical manifestations and early diagnosis of Sjögren syndrome.干燥综合征的临床表现与早期诊断
Arch Intern Med. 2004 Jun 28;164(12):1275-84. doi: 10.1001/archinte.164.12.1275.

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