Progressive myelin oligodendrocyte glycoprotein-associated demyelination mimicking leukodystrophy.

Multiple sclerosis (Houndmills, Basingstoke, England) Pub Date : 2022-08-01 Epub Date: 2022-06-23 DOI:10.1177/13524585221090737

Emily Gibbons, Daniel Whittam, Kariem Elhadd, Maneesh Bhojak, Nitika Rathi, Shivaram Avula, Anu Jacob, Michael Griffiths, Saif Huda

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Abstract

Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) may be associated with relapsing disease, but clinical progression independent of relapse activity is rare.

Objectives: To report progressive disease in a patient with MOGAD.

Methods: A single retrospective case report.

Results: At 4 years of age, the patient had a single episode of acute disseminated encephalomyelitis. She remained well until age 17 years but over the next 9 years developed progressive spastic quadriparesis, cognitive and bulbar dysfunction. Brain imaging showed a leukodystrophy-like pattern of white matter abnormality with contrast enhancement at different time points. Myelin oligodendrocyte glycoprotein (MOG)-IgG was repeatedly positive by live cell-based assay.

Conclusion: Secondary progression may be a rare presentation of MOG-IgG-associated disease.

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进行性髓鞘少突胶质细胞糖蛋白相关脱髓鞘模拟白质营养不良。

背景:髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)可能与疾病复发有关，但独立于复发活动的临床进展是罕见的。目的:报道一例MOGAD患者的进展性疾病。方法:单例回顾性病例报告。结果:在4岁时，患者有一次急性播散性脑脊髓炎发作。直到17岁，她一直保持健康，但在接下来的9年里，她出现了进行性痉挛性四肢瘫、认知和球功能障碍。脑成像显示脑白质异常样样，不同时间点的对比增强。活细胞法检测髓鞘少突胶质细胞糖蛋白igg重复阳性。结论:继发性进展可能是mog - igg相关疾病的罕见表现。

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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Multiple sclerosis (Houndmills, Basingstoke, England)

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