Department of Neurology, Beijing Jishuitan Hospital, Beijing, China.
Department of Neurology, Beijing Jishuitan Hospital, Beijing, China.
Mult Scler Relat Disord. 2018 Apr;21:78-83. doi: 10.1016/j.msard.2018.02.022. Epub 2018 Feb 19.
Short transverse myelitis (STM) is considered uncommon in neuromyelitis optica spectrum disorders (NMOSD). Poor recognition of STM occurring in NMOSD may lead to increased delay in diagnosis and appropriate treatment.
The aim of this study was to assess the frequency and characteristics of STM in Chinese patients with NMOSD.
We enrolled 91 patients with NMOSD based on the 2015 International Consensus Diagnostic Criteria for NMOSD. The patients were divided into STM group and longitudinally extensive transverse myelitis (LETM) group according to the length of initial spinal cord lesions at the initial myelitis manifestation of NMOSD.
Initial STM was observed in 18 patients (18/91, 19.8%). The STM episode was the first manifestation of NMOSD in 9 patients (50%) and preceded by optic neuritis in 3 patients (16.7%), area postrema syndrome in 5(27.8%) and brainstem syndrome in 1(5.6%). Compared to the NMOSD patients with an initial LETM, patients with STM suffered less motor and bowel or bladder disability, had minor EDSS at clinical onset, but suffered earlier relapse (P<.05). Thirteen patients had single short spinal lesion (13/18, 72.2%) and 5 patients had two short lesions. Of the 23 STM lesions, 4 lesions spanned 2.5 vertebral segments, 12 showed a length of continuous 2 vertebral segments, 7 were confined to single vertebral segment. The lesions on axial imaging involved the central grey matter in 61.1% (11/18) patients with STM and in 95.9%(70/73)patients with LETM (P<.05). Both the patients with STM(50%)and LETM (34.2%) had brain lesions.
Initial STM does not exclude consideration of NMOSD diagnosis.
短节段横贯性脊髓炎(STM)被认为在视神经脊髓炎谱系疾病(NMOSD)中不常见。对 NMOSD 中发生的 STM 认识不足可能导致诊断和适当治疗的延迟增加。
本研究旨在评估中国 NMOSD 患者中 STM 的频率和特征。
我们根据 2015 年 NMOSD 的国际共识诊断标准纳入 91 例 NMOSD 患者。根据 NMOSD 初始脊髓炎表现时初始脊髓病变的长度,将患者分为 STM 组和长节段横贯性脊髓炎(LETM)组。
18 例(18/91,19.8%)患者出现初始 STM。9 例(50%)患者的 STM 发作是 NMOSD 的首发表现,3 例(16.7%)患者视神经炎之前发生 STM,5 例(27.8%)患者出现延髓后极综合征,1 例(5.6%)患者出现脑干综合征。与初始 LETM 的 NMOSD 患者相比,STM 患者运动和肠或膀胱功能障碍较少,发病时 EDSS 评分较低,但复发较早(P<.05)。13 例患者有单一短脊髓病变(13/18,72.2%),5 例患者有两个短病变。23 个 STM 病变中,4 个病变跨越 2.5 个椎体节段,12 个病变连续 2 个椎体节段,7 个病变局限于单个椎体节段。轴位成像显示,61.1%(11/18)的 STM 患者和 95.9%(70/73)的 LETM 患者病变累及中央灰质(P<.05)。STM 组(50%)和 LETM 组(34.2%)各有 1 例患者存在脑部病变。
初始 STM 并不能排除 NMOSD 的诊断。