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复发性特发性横贯性脊髓炎的临床特征及预测因素

Clinical characteristics and predictive factors of recurrent idiopathic transverse myelitis.

作者信息

Lee Eun Kyoung, Kim Sooyoung, Sohn Eunhee

机构信息

Department of Neurology, Chungnam National University Sejong Hospital, Sejong, Republic of Korea.

Department of Neurology, Chungnam National University Hospital, Daejeon, Republic of Korea.

出版信息

Front Neurol. 2024 Sep 19;15:1416251. doi: 10.3389/fneur.2024.1416251. eCollection 2024.

Abstract

BACKGROUND

Idiopathic transverse myelitis (iTM) is defined as an inflammatory myelopathy of undetermined etiology, even after a comprehensive workup to identify other possible causes. Generally, the characteristics of recurrent iTM are not clearly defined. This study aimed to identify the clinical characteristics and predictive factors of recurrence in patients with iTM.

METHODS

We retrospectively recruited patients with transverse myelitis (TM) who visited Chungnam National University Hospital between January 2011 and December 2021. We included patients who were followed up for at least 2 years and excluded those diagnosed with multiple sclerosis or neuromyelitis optica spectrum disorder (NMOSD) during the initial episode or follow-up period. Patients with iTM were categorized into two groups: monophasic idiopathic TM (mTM) and recurrent idiopathic TM (rTM). We compared the clinical characteristics and spinal magnetic resonance imaging findings between the two groups.

RESULTS

In total, 167 patients were reviewed, of whom 112 were excluded. Finally, we included 55 patients with iTM. In 55 patients, 11 (20.0%) and 44 (80%) were classified into the rTM and mTM groups, respectively. Male predominance was observed in the iTM, rTM, and mTM groups. The percentage of patients with low vitamin D levels was significantly higher in the rTM group (100.0%) compared with the mTM group (70%) ( = 0.049). In addition, longitudinally extensive transverse myelitis (LETM) was observed more frequently in the rTM group, in 8 of 11 (72.7%) patients, compared with 15 of 44 (34.1%) patients in the mTM group, with the difference being statistically significant ( = 0.020). In multivariate regression analysis, female sex, younger age at onset, low serum vitamin D level (<30 ng/mL), and LETM were risk factors for recurrence. LETM was a significant predictor of relapse in iTM ( = 0.043, odds ratio = 13.408).

CONCLUSION

In this study, the clinical features of mTM and rTM are nearly indistinguishable. In conclusion, >20% of the patients with iTM experience recurrence, and LETM is the most significant risk factor for recurrence. In cases of recurrence, there is a favorable response to immunotherapy, and the prognosis is generally good. Although LETM may be the initial symptom of NMOSD, it may be manifestation of iTM, and in cases of idiopathic LETM, it is important to be mindful of the elevated risk of recurrence. Based on these results, idiopathic rTM has good clinical prognosis and response to immunosuppressive treatment.

摘要

背景

特发性横贯性脊髓炎(iTM)被定义为病因不明的炎症性脊髓病,即便经过全面检查以确定其他可能病因后仍是如此。一般来说,复发性iTM的特征尚未明确界定。本研究旨在确定iTM患者复发的临床特征及预测因素。

方法

我们回顾性招募了2011年1月至2021年12月期间就诊于忠南国立大学医院的横贯性脊髓炎(TM)患者。我们纳入了至少随访2年的患者,并排除了在初始发作期或随访期被诊断为多发性硬化或视神经脊髓炎谱系障碍(NMOSD)的患者。iTM患者被分为两组:单相特发性TM(mTM)和复发性特发性TM(rTM)。我们比较了两组之间的临床特征和脊髓磁共振成像结果。

结果

总共审查了167例患者,其中112例被排除。最终,我们纳入了55例iTM患者。在这55例患者中,分别有11例(20.0%)和44例(80%)被归入rTM组和mTM组。iTM组、rTM组和mTM组均以男性为主。rTM组维生素D水平低的患者百分比(100.0%)显著高于mTM组(70%)(P = 0.049)。此外,rTM组纵向广泛横贯性脊髓炎(LETM)的发生率更高,11例患者中有8例(72.7%),而mTM组44例患者中有15例(34.1%),差异具有统计学意义(P = 0.020)。多因素回归分析显示,女性、发病年龄较小、血清维生素D水平低(<30 ng/mL)和LETM是复发的危险因素。LETM是iTM复发的显著预测因素(P = 0.043,比值比 = 13.408)。

结论

在本研究中,mTM和rTM的临床特征几乎难以区分。总之,超过20%的iTM患者会复发,且LETM是复发的最显著危险因素。在复发病例中,免疫治疗反应良好,预后总体较好。尽管LETM可能是NMOSD的初始症状,但也可能是iTM的表现,在特发性LETM病例中,需注意复发风险升高。基于这些结果,特发性rTM具有良好的临床预后且对免疫抑制治疗有反应。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4cf8/11448116/1008675fa5c9/fneur-15-1416251-g001.jpg

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