Follow-up study on Chinese children with relapsing MOG-IgG-associated central nervous system demyelination

• Published in: Multiple sclerosis and related disorders 2019-02, Vol.28, p.4-10
• Main Authors: Zhou, Ji, Lu, Xiaopeng, Zhang, Yao, Ji, Taoyun, Jin, Yiwen, Xu, Min, Bao, Xinhua, Zhang, Yuehua, Xiong, Hui, Chang, Xingzhi, Jiang, Yuwu, Wu, Ye
• Format: Article
• Language: English
• ISSN: 2211-0348; 2211-0356
• DOI: 10.1016/j.msard.2018.12.001

Some studies have reported clinical features of relapsing MOG-IgG-associated CNS demyelination principally in Caucasians children. It is not clear whether Chinese children share the same phenotype. To delineate the clinical characteristics in Chinese children with relapsing MOG-IgG-associated demyelination. A follow-up study on 23 Children with relapsing MOG-IgG-associated demyelination from two Chinese tertiary hospitals was performed. Phenotypic features at each demyelinating attacks, neuroimaging characteristics, autoimmune antibodies in CSF/serum, response to disease modifying drugs and functional deficits during the disease course were analyzed. The median age at disease onset was 5.38 (2.33-12.75) years. The male to female ratio was 1:1.30. The disease duration was 2.33(1.00-8.92) years at the last follow-up. (1) Clinical phenotypes: ADEM was the most common initial presentation (12/23, 52.17%). In 82 attacks during disease course, ADEM was also the most common phenotype (30/82, 36.59%), followed by ON (24/82, 29.27%). (2) Imaging findings: 57/70 (81.43%) brain MRI scans during acute attacks showed new lesions. The most common location of new lesions in brain was the juxtacortical white matter (45/57, 78.95%). In 46 brain MRI scans with supratentorial white matter lesions, ADEM-like patterns were most common (25/46, 54.35%), and 5/46 (10.87%) scans exhibited leukodystrophy-like patterns. (3) Laboratory examinations: Anti-NMDA receptor IgG in CSF was detected in two patients (2/12, 16.67%), with one patient presented with anti-NMDAR encephalitis associated symptoms. (4) Therapeutic responses and outcomes: In 19 patients treated with disease-modifying drugs (including rituximab, mycophenolate mofetil, azathioprine and so on) longer than 6 months, median annualised relapse rates decreased from 1.71 before treatment to 0.44 during treatment (P