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具有特征性的视网膜萎缩模式可在单次视神经炎发作后区分小儿 MO GAD 和 MS。

Characteristic retinal atrophy pattern allows differentiation between pediatric MOGAD and MS after a single optic neuritis episode.

机构信息

Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, 44791, Bochum, Germany.

Institute of Clinical Neuroimmunology, LMU Hospital, Ludwig-Maximilians Universität München, Munich, Germany.

出版信息

J Neurol. 2022 Dec;269(12):6366-6376. doi: 10.1007/s00415-022-11256-y. Epub 2022 Jul 23.

Abstract

BACKGROUND

Optic neuritis (ON) is the most prevalent manifestation of pediatric multiple sclerosis (MS) and myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in children > 6 years. In this study, we investigated retinal atrophy patterns and diagnostic accuracy of optical coherence tomography (OCT) in differentiating between both diseases after the first ON episode.

METHODS

Patients were retrospectively identified in eight tertial referral centers. OCT, VEP and high/low-contrast visual acuity (HCVA/LCVA) have been investigated > 6 months after the first ON. Prevalence of pathological OCT findings was identified based on data of 144 age-matched healthy controls.

RESULTS

Thirteen MOGAD (10.7 ± 4.2 years, F:M 8:5, 21 ON eyes) and 21 MS (14.3 ± 2.4 years, F:M 19:2, 24 ON eyes) patients were recruited. We observed a significantly more profound atrophy of both peripapillary and macular retinal nerve fiber layer in MOGAD compared to MS (pRNFL global: 68.2 ± 16.9 vs. 89.4 ± 12.3 µm, p < 0.001; mRNFL: 0.12 ± 0.01 vs. 0.14 ± 0.01 mm, p < 0.001). Neither other macular layers nor P100 latency differed. MOGAD developed global atrophy affecting all peripapillary segments, while MS displayed predominantly temporal thinning. Nasal pRNFL allowed differentiation between both diseases with the highest diagnostic accuracy (AUC = 0.902, cutoff < 62.5 µm, 90.5% sensitivity and 70.8% specificity for MOGAD). OCT was also substantially more sensitive compared to VEP in identification of ON eyes in MOGAD (pathological findings in 90% vs. 14%, p = 0.016).

CONCLUSION

First MOGAD-ON results in a more severe global peripapillary atrophy compared to predominantly temporal thinning in MS-ON. Nasal pRNFL allows differentiation between both diseases with the highest accuracy, supporting the additional diagnostic value of OCT in children with ON.

摘要

背景

视神经炎(ON)是儿童多发性硬化症(MS)和髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)中最常见的表现,发生于> 6 岁的儿童。在这项研究中,我们调查了儿童首次发生 ON 后,通过光学相干断层扫描(OCT)区分这两种疾病的视网膜萎缩模式和诊断准确性。

方法

在 8 家三级转诊中心回顾性地确定了患者。在首次 ON 后> 6 个月,对 OCT、VEP 和高/低对比度视力(HCVA/LCVA)进行了检查。根据 144 名年龄匹配的健康对照者的数据,确定了病理性 OCT 发现的患病率。

结果

共纳入 13 例 MOGAD(10.7±4.2 岁,F:M 8:5,21 只 ON 眼)和 21 例 MS(14.3±2.4 岁,F:M 19:2,24 只 ON 眼)患者。我们观察到 MOGAD 的视盘周围和黄斑视网膜神经纤维层萎缩程度明显比 MS 更严重(pRNFL 全局:68.2±16.9 比 89.4±12.3µm,p<0.001;mRNFL:0.12±0.01 比 0.14±0.01mm,p<0.001)。其他黄斑层和 P100 潜伏期没有差异。MOGAD 发展为影响所有视盘周围节段的全局萎缩,而 MS 则表现为主要的颞侧变薄。鼻侧 pRNFL 对两种疾病的鉴别具有最高的诊断准确性(AUC=0.902,截断值<62.5µm,对 MOGAD 的敏感性为 90.5%,特异性为 70.8%)。与 VEP 相比,OCT 在识别 MOGAD 中的 ON 眼方面也具有更高的敏感性(病理性发现 90%比 14%,p=0.016)。

结论

首次 MOGAD-ON 导致的视盘周围萎缩比 MS-ON 更严重,呈全球性,且主要为颞侧变薄。鼻侧 pRNFL 对两种疾病的鉴别具有最高的准确性,支持 OCT 在 ON 患儿中的额外诊断价值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4caf/9618526/753e93f28209/415_2022_11256_Fig1_HTML.jpg

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