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儿童髓鞘少突胶质细胞糖蛋白抗体相关性疾病(MOGAD)脑 MRI 病变的演变及其与疾病过程的相关性。

Evolution of brain MRI lesions in paediatric myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and its relevance to disease course.

机构信息

Department of Neuroinflammation, UCL Institute of Neurology, University College London, London, UK.

Department of Neurology, Great Ormond Street Hospital for Children, London, UK.

出版信息

J Neurol Neurosurg Psychiatry. 2024 Apr 12;95(5):426-433. doi: 10.1136/jnnp-2023-332542.

DOI:10.1136/jnnp-2023-332542
PMID:37979966
Abstract

BACKGROUND

Lesion resolution is often observed in children with myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and asymptomatic lesions are less commonly reported in MOGAD than in multiple sclerosis (MS).

OBJECTIVE

We aimed to evaluate brain MRI changes over time in paediatric MOGAD.

METHODS

Retrospective study in eight UK paediatric neuroscience centres. Acute brain MRI and available follow-up MRIs were reviewed. Predictors for lesion dynamic were evaluated using multivariable regression and Kaplan-Meier survival analyses were used to predict risk of relapse, disability and MOG-Ab status.

RESULTS

200 children were included (MOGAD 97; MS 103). At first MRI post attack, new symptomatic and asymptomatic lesions were seen more often in MS versus MOGAD (52/103 vs 28/97; p=0.002 and 37/103 vs 11/97; p<0.001); 83% of patients with MOGAD showed at least one lesion's resolution at first follow-up scan, and 23% had normal MRI. Only 1 patient with MS had single lesion resolution; none had normal MRI. Disappearing lesions in MOGAD were seen in 40% after the second attack, 21% after third attack and none after the fourth attack.New lesions at first follow-up scan were associated with increased likelihood of relapse (p=0.02) and persistent MOG-Ab serostatus (p=0.0016) compared with those with no new lesions. Plasma exchange was associated with increased likelihood of lesion resolution (p=0.01). Longer time from symptom onset to steroids was associated with increased likelihood of new lesions; 50% increase at 20 days (p=0.01).

CONCLUSIONS

These striking differences in lesion dynamics between MOGAD and MS suggest greater potential to repair. Early treatment with steroids and plasma exchange is associated with reduced likelihood of new lesions.

摘要

背景

髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)患儿常出现病灶消退,无症状病灶较多发性硬化(MS)少见。

目的

评估儿童 MOGA 相关疾病的脑 MRI 变化。

方法

回顾性研究 8 家英国儿科神经科学中心。对急性脑 MRI 和现有的随访 MRI 进行了回顾。采用多变量回归评估病灶动态的预测因素,采用 Kaplan-Meier 生存分析预测复发、残疾和 MOG-Ab 状态的风险。

结果

纳入 200 名患儿(MOGAD97 例,MS103 例)。在发病后首次 MRI 中,MS 患儿的新发症状性和无症状性病灶较 MOGAD 患儿更常见(52/103 例比 28/97 例;p=0.002 和 37/103 例比 11/97 例;p<0.001);83%的 MOGAD 患儿在首次随访扫描中至少有一处病灶消退,23%的患儿 MRI 正常。仅有 1 例 MS 患儿出现单一病灶消退,无 MRI 正常。MOGAD 患儿的病灶在第 2 次发作后 40%、第 3 次发作后 21%、第 4 次发作后均消失。首次随访时出现新病灶与复发的可能性增加(p=0.02)和持续的 MOG-Ab 血清阳性(p=0.0016)相关,与无新病灶的患儿相比。与无新病灶的患儿相比,血浆置换与病灶消退的可能性增加相关(p=0.01)。从症状出现到类固醇治疗的时间越长,新病灶的可能性越大;20 天增加 50%(p=0.01)。

结论

MOGAD 和 MS 之间病灶动态的这些显著差异表明有更大的修复潜力。早期应用类固醇和血浆置换治疗与新病灶减少相关。

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引用本文的文献

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Pediatr Radiol. 2025 Apr;55(4):699-720. doi: 10.1007/s00247-024-06023-2. Epub 2024 Sep 7.
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Neurology. 2024 May 28;102(10):e209303. doi: 10.1212/WNL.0000000000209303. Epub 2024 May 6.