关键词: Longitudinally extensive transverse myelitis Magnetic resonance imaging Neuromyelitis optica spectrum disorder Transverse myelitis

Mesh : Humans Neuromyelitis Optica / diagnostic imaging complications immunology Myelitis, Transverse / diagnostic imaging immunology Female Adult Male Middle Aged Aquaporin 4 / immunology Magnetic Resonance Imaging Young Adult Retrospective Studies Spinal Cord / diagnostic imaging pathology Autoantibodies / blood

来  源:   DOI:10.1016/j.msard.2024.105666

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.
METHODS: 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.
CONCLUSIONS: 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障碍(NMOSD)的自身免疫机制的重要靶标之一。很少,NMOSD会对整个脊髓造成损伤,从子宫颈到延髓圆锥,这在现有文献中没有被定性。
方法:我们回顾了医疗记录,人口统计信息,2011年1月至2023年1月,174名NMOSD患者的磁共振成像(MRI)序列被送往伊斯法罕多发性硬化中心,以发现患有全脊横贯性脊髓炎(TM)的患者。
结果:5例患者存在全脊髓TM(2.9%)。3例患者水通道蛋白4(AQP4)抗体呈血清阳性;髓鞘少突胶质细胞糖蛋白抗体(MOGIgG)检测均为阴性。下肢无力是最常见的临床表现。两名患者出现视神经炎;一名患者报告有恶心和呕吐发作。这些病人,总的来说,与其他NMOSD患者相比,获得了更高的扩展残疾状态量表(EDSS)评分。
结论:整个脊髓TM在NMOSD中很少见,这与疾病的严重程度更高和结果更差密切相关。抗AQP4抗体在NMOSD脊髓炎程度中的作用尚待研究。
公众号