Factors associated with functional status at presentation in patients with longitudinally extensive transverse myelitis.

IF 3.5 2区医学 Q2 GENETICS & HEREDITY

Orphanet Journal of Rare Diseases Pub Date : 2025-09-02 DOI:10.1186/s13023-025-03999-4

Nisa Vorasoot, Pilantana Saichua, Prapassara Sirikarn, Narongrit Kasemsap, Kannikar Kongbunkiat, Somsak Tiamkao, Verajit Chotmongkol, Kittisak Sawanyawisuth

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引用次数: 0

Abstract

Background: Longitudinally extensive transverse myelitis (LETM) is a rare neurological disease. A case series evaluated the predictors of a long-term clinical outcome. However, there is limited data on predictors of functional status at presentation. Therefore, this study aimed to find clinical factors predictive of poor clinical presentation in Asian patients with LETM.

Methods: This was a cross-sectional, retrospective analytical study. The inclusion criteria were adult patients with an age of 18 years or more who met the criteria of LETM. Clinical factors of eligible patients were recorded. Eligible patients were categorized into two groups based on the Expanded Disability Status Scale (EDSS) at the time of presentation; 6 or more vs. 0-5.5. A predictive model for the EDSS score at the time of presentation was created by the logistic regression analysis.

Results: There were 40 patients with LETM in the study. Of those, 31 patients (77.50%) had an EDSS of 6 or more. There were two factors in the model predictive of the EDSS at presentation of 6 or more; thoracic lesion and complete lesion. Only a complete lesion was independently associated with an EDSS at presentation of 6 or more with an adjusted odds ratio of 15.202 (95% confidence interval of 2.028, 113.968; p value = 0.008).

Conclusions: The complete cord lesion was independently associated with severe functional status in patients with LETM at presentation.

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与纵向广泛横断面脊髓炎患者出现时功能状态相关的因素。

背景：纵向广泛性横贯脊髓炎是一种罕见的神经系统疾病。一个病例系列评估了长期临床结果的预测因素。然而，有有限的数据预测功能状态的表现。因此，本研究旨在寻找预测亚洲LETM患者不良临床表现的临床因素。方法：这是一项横断面回顾性分析研究。纳入标准为年龄在18岁及以上且符合LETM标准的成年患者。记录符合条件的患者的临床因素。根据就诊时的扩展残疾状态量表（EDSS）将符合条件的患者分为两组；6或以上vs. 0-5.5。通过逻辑回归分析，建立了报告时EDSS评分的预测模型。结果：本组共40例LETM患者。其中，31例（77.50%）患者的EDSS≥6。在模型中有两个因子可以预测出现6分及以上的EDSS；胸部病变和完全病变。只有完整病变与6分及以上的EDSS独立相关，校正优势比为15.202（95%可信区间为2.028,113.968；p值= 0.008）。结论：完全性脊髓损伤与LETM患者的严重功能状态独立相关。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Orphanet Journal of Rare Diseases 医学-医学：研究与实验

CiteScore

6.30

自引率

8.10%

发文量

418

审稿时长

4-8 weeks

期刊介绍： Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.