Clinical Characteristics of Patients With Neuromyelitis Optica Spectrum Disorders With Early Onset

Area of Science:

• Neurology
• Immunology
• Pediatrics

Background:

• Neuromyelitis Optica Spectrum Disorder (NMOSD) is a severe autoimmune disease.
• NMOSD typically affects middle-aged adults but can occur in children and adolescents.
• Early-onset NMOSD may have distinct clinical characteristics and prognoses.

Purpose of the Study:

• To analyze the clinical data of 36 Brazilian patients with NMOSD who experienced disease onset before age 21.
• To investigate the impact of early-onset NMOSD on disease severity and long-term outcomes.
• To compare the course of NMOSD in patients with onset before 12 years versus those with onset between 12-21 years.

Main Methods:

• Retrospective assessment of medical records from 14 specialized centers in Brazil.
• Inclusion criteria: patients diagnosed with NMOSD with onset before age 21.
• Data collected included clinical presentation, disease course, disability accumulation, and mortality.

Main Results:

• The course of NMOSD was significantly worse in patients with disease onset before the age of 12 years.
• Gender and ethnic background did not appear to influence disability accumulation.
• Over a median follow-up of 8 years, 14% of patients with onset before 21 years died.

Conclusions:

• Early-onset NMOSD, particularly before age 12, is associated with a more severe disease course.
• NMOSD in pediatric and adolescent populations requires careful monitoring and management.
• This study contributes to understanding the long-term outcomes of early-onset NMOSD in Brazil.