Pediatric-Onset Neuromyelitis Optica Spectrum Disorder in Isfahan: Insights from a Cross-Sectional Study.

Abstract

Objectives: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune demyelinating disease of the central nervous system. While NMOSD predominantly affects adults, pediatric-onset NMOSD (PONMOSD) cases are increasingly recognized, necessitating a better understanding of the disease in this population. This study aims to provide a comprehensive insight into the manifestations and management of PONMOSD.

Material & methods: This study was conducted at the Isfahan MS Clinic, Iran, recruiting 182 NMOSD patients between March 2021 and March 2022. Board-certified neurologists performed diagnosis and examination, applying the 2015 NMOSD diagnostic criteria. Clinical data were collected and analyzed, including demographic information, onset symptoms, family history, treatment, and MRI findings.

Results: Eighteen patients with PONMOSD (9.9% of the total) were identified. The study cohort had a female-to-male ratio 2.6:1, with the most common onset symptoms being optic neuritis (ON) and transverse myelitis (TM). AQP4-Ab was positive in 66.7% of the patients, with longitudinally extensive transverse myelitis (LETM) being the most common MRI finding. Azathioprine and Rituximab were the most commonly used treatments in patients, respectively. Treatment response was generally favorable, with most patients responding to therapy.

Conclusion: The present study provides valuable insights into the clinical characteristics and management of pediatric-onset NMOSD. Despite challenges in diagnosis and treatment, early recognition and appropriate management strategies can lead to improved outcomes in this population. Further research is needed to optimize diagnostic criteria and therapeutic approaches for pediatric NMOSD.