A nation-wide survey of Japanese pediatric MOG antibody-associated diseases.

Abstract

OBJECTIVE:

To elucidate the clinical characteristics of Japanese pediatric patients with acquired demyelinating diseases (ADS), positive for myelin oligodendrocyte glycoprotein antibody (MOG-IgG), we conducted a nation-wide survey.

METHODS:

Information about pediatric patients under 18 years old with ADS was solicited with surveys sent to 323 facilities. In an initial survey, we asked whether the center had any patients with ADS, and the MOG-IgG serostatus of the patients. In a follow-up survey, we requested more precise information on patients with ADS.

RESULTS:

Initial survey: 263 replies providing information on 175 patients were received. MOG-IgG were examined in 78 patients and 54 of those (69%) were positive for MOG-IgG. Follow-up survey: The characteristic involvement was optic neuritis, with visual disturbance and optic pain as characteristic symptoms. The relapse rate was 44% in patients positive for MOG-IgG, which was higher than that in seronegative patients (38%). For acute phase treatments, corticosteroid (CS), plasma exchange, and intravenous immunoglobulin (IVIG) were useful. To prevent relapse, CS, intermittent IVIG, immunosuppressants, and monoclonal antibodies were useful, but the efficacies of disease modifying drugs were uncertain. Sequelae such as visual disturbance, cognitive impairment, motor dysfunction, and epilepsy were observed in 11% of patients with MOG-IgG.

CONCLUSIONS:

MOG antibody-associated diseases were found to be common among pediatric ADS patients. Since a variety of sequelae were observed in these patients, it is important to identify the appropriate treatment to ensure the best outcome. The presence of the MOG autoantibody should be taken into consideration as part of the diagnostic criteria for pediatric ADS.

• Authors: Kohji Azumagawa, Ichiro Nakashima, Kimihiko Kaneko, Hiroyuki Torisu, Yasunari Sakai, Ryutaro Kira, Hiroshi Sakuma, Keiko Tanaka, Yasushi Shigeri, Yoshie Tanaka, Hideto Nakajima, Shuichi Shimakawa, Hiroshi Tamai
• Journal: Brain & development (Brain Dev) Vol. 43 Issue 6 Pg. 705-713 (Jun 2021) ISSN: 1872-7131 [Electronic] Netherlands
• PMID: 33610339 (Publication Type: Journal Article)
• Copyright: Copyright © 2021 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Chemical References

• AQP4 protein, human
• Aquaporin 4
• Autoantibodies
• MOG protein, human
• Myelin-Oligodendrocyte Glycoprotein

Topics

• Adolescent
• Aquaporin 4 (immunology)
• Autoantibodies (blood)
• Child
• Child, Preschool
• Cognitive Dysfunction (epidemiology, etiology)
• Demyelinating Autoimmune Diseases, CNS (blood, complications, epidemiology, immunology)
• Epilepsy (epidemiology, etiology)
• Female
• Health Surveys
• Humans
• Japan (epidemiology)
• Male
• Movement Disorders (epidemiology, etiology)
• Myelin-Oligodendrocyte Glycoprotein (immunology)
• Optic Neuritis (blood, complications, epidemiology, immunology)
• Recurrence
• Vision Disorders (epidemiology, etiology)