Kosior N, Perrier R L, Casserly C, Morrow S A, Racosta J M
Department of Clinical Neurological Sciences, University of Western Ontario, University Hospital 339 Windermere Road, London Ontario N6A 5A5 Canada.
Discipline of Medicine, Area of Neurology, Memorial University of Newfoundland, Canada.
Mult Scler Relat Disord. 2024 Dec;92:105941. doi: 10.1016/j.msard.2024.105941. Epub 2024 Oct 15.
Anti-myelin oligodendrocyte glycoprotein associated disease (MOGAD) and neuromyelitis optica spectrum disease (NMOSD) are antibody mediated diseases characterized by neurological symptoms including recurrent relapses of optic neuritis and/or myelitis, as well as other less frequent syndromes. The current treatment for acute attacks of NMOSD/MOGAD are based on clinical studies for other demyelinating diseases(i.e. Multiple Sclerosis). In NMOSD, high dose corticosteroids (HDS) are considered the standard first line therapy, with emerging evidence supporting the use of plasmapheresis (PLEX) as an acute therapy. In MOGAD, being a relatively new clinical syndrome, the consensus on acute treatments is yet to be reached. The objective of our study was to assess the efficacy of treatment regimens (no treatment vs. HDS vs. HDS and PLEX) on disability outcomes in persons with NMOSD and MOGAD-optic neuritis and myelitis.
We retrospectively extracted data from the MuSicaL-NeMo database using a mixed Natural Language Processing followed by investigators verification. We assessed the change in Expanded Disability Status Scale (EDSS) and Visual Acuity (VA) following HDS and PLEX, in persons with MOGAD and NMOSD following myelitis and optic neuritis. We used the novel statistical measure Wilcoxon-Mann-Whitney Odd (WMW-Odd) to calculate the change through all the spectrum of each ordinal scale (VA and EDSS).
Eleven myelitis and 12 optic neuritis in 22 persons with MOGAD and 30 myelitis and 12 optic neuritis in 20 persons with NMOSD were included(15 Aquaporin-4 seropositive). In persons with MOGAD-optic neuritis the group receiving HDS had a WMW-Odd of 15.33(p ≤ 0.001), however those not receiving treatment also tended to improve (WMW-Odd=3.17, p = 0.06). NMOSD-optic neuritis treated with HDS only improve 33.3 % of the times (p=NS). Persons with MOGAD-myelitis receiving HDS significantly improved (WMW-Odd=7.33, p = 0.002). Persons with NMOSD-myelitis treated with HDS had an WMW-Odd of 2.56 (p = 0.002) and those treated with PLEX plus HDS (PLEX+), had similar WMW-Odd of 2.51 (p = 0.03). When correcting for disease severity by restricting inclusion to persons with NMOSD with EDSS≥4, both treatments showed a higher WMW-Odd, however the group receiving HDS continued to show higher WMW-Odd than the PLEX+ group(WMW-Odd= 3.75, p = 0.002 vs. WMW-Odd =3.05, p = 0.02, respectvely) CONCLUSION: Our study suggests that persons with MOGAD-optic neuritis improve without acute treatments, however they have very marked improvement when using HDS, as previously suggested. Patient with MOGAD-myelitis are also very responsive to HDS, however, as compared to MOGAD-optic neuritis, they displayed less improvement, if not treated. In the NMOSD group the use of PLEX in addition to HDS did not demonstrate any significant difference in EDSS outcomes. Contrary to previous suggestions, when adjusting for group differences (by only including EDSS ≥4), the use of HDS and PLEX+ did not show better results than the group using HDS.
抗髓鞘少突胶质细胞糖蛋白相关疾病（MOGAD）和视神经脊髓炎谱系疾病（NMOSD）是抗体介导的疾病，其特征为出现包括视神经炎和/或脊髓炎反复复发以及其他较罕见综合征在内的神经症状。目前针对NMOSD/MOGAD急性发作的治疗是基于针对其他脱髓鞘疾病（即多发性硬化症）的临床研究。在NMOSD中，大剂量皮质类固醇（HDS）被视为标准的一线治疗方法，并且越来越多的证据支持使用血浆置换（PLEX）作为急性治疗手段。在MOGAD方面，作为一种相对较新的临床综合征，对于急性治疗的共识尚未达成。我们研究的目的是评估治疗方案（不治疗 vs. HDS vs. HDS联合PLEX）对患有NMOSD和MOGAD - 视神经炎及脊髓炎患者残疾结局的疗效。
我们使用混合自然语言处理技术从MuSicaL - NeMo数据库中回顾性提取数据，随后由研究人员进行核实。我们评估了MOGAD和NMOSD患者在脊髓炎和视神经炎发作后接受HDS和PLEX治疗后扩展残疾状态量表（EDSS）和视力（VA）的变化。我们使用新颖的统计量Wilcoxon - Mann - Whitney Odd（WMW - Odd）来计算每个有序量表（VA和EDSS）全范围内的变化。
纳入了22例MOGAD患者中的11例脊髓炎和12例视神经炎，以及20例NMOSD患者中的30例脊髓炎和12例视神经炎（15例水通道蛋白4血清阳性）。在患有MOGAD - 视神经炎的患者中，接受HDS治疗的组WMW - Odd为15.33（p≤0.001），然而未接受治疗的患者也有改善趋势（WMW - Odd = 3.17，p = 0.06）。仅接受HDS治疗的NMOSD - 视神经炎患者改善的比例仅为33.3%（p =无统计学意义）。患有MOGAD - 脊髓炎且接受HDS治疗的患者有显著改善（WMW - Odd = 7.33，p = 0.002）。患有NMOSD - 脊髓炎且接受HDS治疗的患者WMW - Odd为2.56（p = 0.002），而接受PLEX加HDS（PLEX +）治疗的患者WMW - Odd类似，为2.51（p = 0.03）。当通过将纳入范围限制为EDSS≥4的NMOSD患者来校正疾病严重程度时，两种治疗方法均显示出更高的WMW - Odd，然而接受HDS治疗的组WMW - Odd继续高于PLEX +组（分别为WMW - Odd = 3.75，p = 0.002 vs. WMW - Odd = 3.05，p = 0.02）
我们的研究表明，患有MOGAD - 视神经炎的患者在未进行急性治疗的情况下也会改善，然而如先前所示，使用HDS时他们会有非常显著的改善。患有MOGAD - 脊髓炎的患者对HDS也非常敏感，然而，与MOGAD - 视神经炎相比，如果不进行治疗，他们的改善程度较小。在NMOSD组中，除HDS外使用PLEX在EDSS结局方面未显示出任何显著差异。与先前的观点相反，当调整组间差异（仅纳入EDSS≥4的患者）时，使用HDS和PLEX +的组并未显示出比使用HDS的组更好的结果。
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