Neuromyelitis Optica Spectrum Disorder in a Pediatric Patient

Literature Review with Case Report

Resumo

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a severe autoimmune astrocytopathy of the central nervous system (CNS) with secondary demyelination, often associated with the anti-aquaporin-4 (anti-AQP4) antibody. Approximately 5–10% of NMOSD cases begin before the age of 18. However, the scarcity of available studies hampers a comprehensive understanding of pediatric NMOSD, particularly regarding treatment.

Objectives: To present a case report of a pediatric patient with NMOSD and to conduct an integrative literature review on treatments already used in pediatric patients, as well as ongoing clinical trials in this population.

Case report: We describe the case of an 11-year-old female patient who presented with NMOSD.

Methods: This is a case report and integrative literature review conducted between October 2024 and January 2025 using the PubMed, Web of Science, Embase, Scopus, EBSCO, LILACS, and SciELO databases.

Results: Eight articles were included in this study after a series of screenings. Additionally, four clinical trials involving pediatric patients were found to be ongoing or recently completed in clinicaltrials.gov.

Conclusion: The management of pediatric NMOSD requires a stepwise approach in the acute phase and effective immunosuppression for relapse prevention. Satralizumab is indicated for patients aged 12 years and older, Rituximab proved to be the most effective option; however, its limited availability in the Brazilian public health system (SUS) hinders its application. The clinical case highlights the importance of early diagnosis and appropriate control to prevent sequelae and minimize treatment-related adverse effects.