进行性髓鞘少突胶质细胞糖蛋白相关性脱髓鞘病，类似脑白质营养不良。
Progressive myelin oligodendrocyte glycoprotein-associated demyelination mimicking leukodystrophy.
机构信息
National Neuromyelitis Optica Spectrum Disorders Service, The Walton Centre NHS Foundation Trust, Liverpool, UK.
Department of Neurology, Salford Royal NHS Foundation Trust, Salford, UK.
出版信息
Mult Scler. 2022 Aug;28(9):1481-1484. doi: 10.1177/13524585221090737. Epub 2022 Jun 23.
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.
背景
髓鞘少突胶质细胞糖蛋白抗体相关性疾病（MOGAD）可能与复发疾病相关，但与复发活动无关的临床进展较为罕见。
目的
报告 1 例 MOGAD 患者的进行性疾病。
方法
单例回顾性病例报告。
结果
4 岁时，患者发生了单次急性播散性脑脊髓炎。她一直保持良好，直到 17 岁，但在接下来的 9 年中，她逐渐出现痉挛性四肢瘫痪、认知和球部功能障碍。脑部影像学显示出一种白质异常的脱髓鞘样模式，不同时间点有对比增强。髓鞘少突胶质细胞糖蛋白（MOG）-IgG 反复通过活细胞检测呈阳性。
结论
继发性进展可能是 MOG-IgG 相关性疾病的一种罕见表现。
Zotero 插件