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视神经脊髓炎谱系疾病中的全横贯性脊髓炎。

Whole spinal transverse myelitis in neuromyelitis optica spectrum disorder.

机构信息

Department of Neurosurgery, Alzahra University Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.

Alzahra Research Institute, Alzahra University Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.

出版信息

Mult Scler Relat Disord. 2024 Jul;87:105666. doi: 10.1016/j.msard.2024.105666. Epub 2024 May 8.

Abstract

BACKGROUND

Spinal cord is one of the prominent targets of autoimmune mechanisms in Neuromyelitis Optica Spectrum Disorder (NMOSD). Rarely, NMOSD causes damage to the entire length of the spinal cord, from cervical segments to conus medullaris, which has not been characterized in the existing literature.

MATERIAL AND METHOD

We reviewed medical records, demographic information, and magnetic resonance imaging (MRI) sequences of 174 NMOSD patients from January 2011 to January 2023 who were admitted to Isfahan Multiple Sclerosis center to find patients with whole spinal transverse myelitis (TM).

RESULTS

Whole spinal TM was present in five patients (2.9 %). Three patients were seropositive for Aquaporin-4 (AQP4) antibody; Myelin Oligodendrocyte Glycoprotein antibody (MOG IgG) tested negative for all of them. Lower limb weakness was the most frequent clinical complaint. Two patients presented with optic neuritis; One patient reported having episodes of nausea and vomiting. These patients, overall, yielded a higher expanded disability status scale (EDSS) score than the other NMOSD patients.

CONCLUSION

Whole spinal TM is a rare finding in NMOSD, which is strongly associated with a higher severity and a worse outcome of the disease. The role of anti-AQP4 antibodies in the extent of myelitis in NMOSD has yet to be investigated.

摘要

背景

脊髓是视神经脊髓炎谱系疾病(NMOSD)中自身免疫机制的主要靶标之一。NMOSD 很少导致整个脊髓(从颈段到脊髓圆锥)受损,这在现有文献中尚未得到描述。

材料和方法

我们回顾了 2011 年 1 月至 2023 年 1 月期间在伊斯法罕多发性硬化症中心住院的 174 名 NMOSD 患者的病历、人口统计学信息和磁共振成像(MRI)序列,以寻找患有全脊髓横贯性脊髓炎(TM)的患者。

结果

全脊髓 TM 见于 5 名患者(2.9%)。3 名患者抗水通道蛋白-4(AQP4)抗体阳性;所有患者的髓鞘少突胶质细胞糖蛋白抗体(MOG IgG)检测均为阴性。下肢无力是最常见的临床症状。2 名患者出现视神经炎;1 名患者报告有恶心和呕吐症状。这些患者的扩展残疾状态量表(EDSS)评分总体上高于其他 NMOSD 患者。

结论

全脊髓 TM 在 NMOSD 中是一种罕见的表现,与疾病的严重程度和预后较差密切相关。抗 AQP4 抗体在 NMOSD 中髓炎程度中的作用尚待研究。

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