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复发抗体阴性且具有视神经脊髓炎谱系疾病特征的患者:颈髓内乙酰天门冬氨酸水平的差异表明存在不同的潜在发病机制。

Relapsing antibody-negative patients with features of neuromyelitis optica spectrum disorders: Differences in -acetylaspartate level in the cervical spinal cord indicate distinct underlying processes.

机构信息

Laboratory of Brain Imaging, Neurobiology Center, Nencki Institute of Experimental Biology, Polish Academy of Sciences, Warsaw, Poland.

Wellcome Centre for Integrative Neuroimaging, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.

出版信息

Mult Scler. 2022 Dec;28(14):2221-2230. doi: 10.1177/13524585221115304. Epub 2022 Aug 15.

DOI:10.1177/13524585221115304
PMID:35971567
Abstract

BACKGROUND

Due to lack of biomarkers, antibody-negative patients with features of neuromyelitis optica spectrum disorders (NMOSD) are among the most challenging to diagnose and treat. Using unsupervised clustering, we recently identified 'MS-like', 'spinal MS-like', 'classic NMOSD-like' and 'NMOSD-like with brain involvement' subgroups in this cohort.

OBJECTIVE

We used magnetic resonance spectroscopy (MRS) to examine differences in the level of key metabolites in the spinal cord between the four identified subgroups.

METHODS

Twenty-five relapsing antibody-negative patients with NMOSD features classified by the unsupervised algorithm to one of the subgroups underwent a prospective cervical spinal cord MRS. Spectra from 16 patients fulfilled quality criteria and were included in the analysis.

RESULTS

Total -acetylaspartate (tNAA), but not total choline (tCho) or myo-inositol (Ins), was significantly different between the four subgroups ( = 0.03). In particular, tNAA was 47.8% lower in the 'MS-like' subgroup as compared with the 'classic NMOSD-like' subgroup ( = 0.02). While we found a negative overall correlation between tNAA and disability score ( = -0.514,  = 0.04) in the whole cohort, the disability score did not differ significantly between the subgroups to explain subgroup differences in tNAA level.

CONCLUSIONS

Significant differences in the cervical spinal cord tNAA measurements confirm that the previously identified clinico-radiologic subgroups contain patients with distinct underlying disease processes.

摘要

背景

由于缺乏生物标志物,具有视神经脊髓炎谱系疾病(NMOSD)特征的抗体阴性患者是诊断和治疗最具挑战性的患者之一。最近,我们使用无监督聚类方法在该队列中发现了“MS 样”、“脊髓 MS 样”、“经典 NMOSD 样”和“伴有脑受累的 NMOSD 样”四个亚组。

目的

我们使用磁共振波谱(MRS)检查这四个亚组之间脊髓中关键代谢物水平的差异。

方法

25 例经无监督算法分类为亚组之一的复发性抗体阴性 NMOSD 特征患者接受前瞻性颈髓 MRS 检查。16 例患者的光谱符合质量标准并纳入分析。

结果

总乙酰天冬氨酸(tNAA),而不是总胆碱(tCho)或肌醇(Ins),在四个亚组之间有显著差异(=0.03)。特别是,“MS 样”亚组的 tNAA 比“经典 NMOSD 样”亚组低 47.8%(=0.02)。虽然我们发现整个队列中 tNAA 与残疾评分之间存在负相关(=−0.514,=0.04),但残疾评分在亚组之间没有显著差异,无法解释 tNAA 水平的亚组差异。

结论

颈髓 tNAA 测量的显著差异证实了先前确定的临床放射学亚组包含具有不同潜在疾病过程的患者。

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