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A nation-wide survey of Japanese pediatric MOG antibody-associated diseases
Kohji Azumagawa1, Ichiro Nakashima2, Kimihiko Kaneko3
1Department of Pediatrics, Seikeikai Hospital, Osaka, Japan; Department of Chemistry, Wakayama Medical University, Wakayama, Japan.
Insights
Myelin oligodendrocyte glycoprotein antibody-associated diseases are common in Japanese children with acquired demyelinating diseases. Early identification and appropriate treatment are crucial for managing MOG-IgG positive pediatric patients and preventing long-term sequelae.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Acquired demyelinating diseases (ADS) in children can have various causes.
- Myelin oligodendrocyte glycoprotein antibody (MOG-IgG) is increasingly recognized as a significant factor in pediatric neurological disorders.
Purpose of the Study:
- To determine the prevalence and clinical characteristics of MOG-IgG positive ADS in Japanese pediatric patients.
- To evaluate treatment efficacies and identify sequelae in this population.
Main Methods:
- A nationwide survey of 323 facilities was conducted to identify pediatric ADS patients.
- Initial surveys collected information on ADS presence and MOG-IgG status, followed by detailed data collection in a follow-up survey.
Main Results:
- Of 175 pediatric ADS patients identified, 69% tested positive for MOG-IgG.
- Optic neuritis was a common presentation. Relapse occurred in 44% of MOG-IgG positive patients.
- Corticosteroids, plasma exchange, and IVIG were effective acute treatments; relapse prevention involved various immunotherapies, with uncertain efficacy for disease-modifying drugs. Sequelae were noted in 11%.
Conclusions:
- MOG antibody-associated diseases are prevalent in pediatric ADS patients in Japan.
- Appropriate treatment strategies are essential to minimize sequelae and improve outcomes.
- MOG autoantibody testing should be considered in the diagnostic workup of pediatric ADS.
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.
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