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

Area of Science:

• Neuroimmunology
• Pediatric Neurology
• Demyelinating Diseases

Background:

• Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare autoimmune CNS demyelinating disease.
• While predominantly affecting adults, pediatric-onset NMOSD (PONMOSD) is increasingly recognized.
• Understanding PONMOSD manifestations and management is crucial for this population.

Purpose of the Study:

• To provide comprehensive insights into the clinical characteristics of PONMOSD.
• To analyze the management strategies employed for PONMOSD.
• To evaluate treatment responses in pediatric NMOSD patients.

Main Methods:

• Retrospective analysis of 182 NMOSD patients at Isfahan MS Clinic (March 2021-March 2022).
• Diagnosis based on 2015 NMOSD criteria by board-certified neurologists.
• Data collection included demographics, onset symptoms, family history, treatment, and MRI findings.

Main Results:

• Eighteen (9.9%) patients had PONMOSD.
• Common onset symptoms: optic neuritis (ON) and transverse myelitis (TM).
• 66.7% tested positive for AQP4-Ab; longitudinally extensive transverse myelitis (LETM) was the most common MRI finding. Azathioprine and Rituximab were primary treatments with favorable responses.

Conclusions:

• This study offers valuable insights into PONMOSD clinical features and management.
• Early recognition and appropriate strategies improve outcomes despite diagnostic and treatment challenges.
• Further research is needed to optimize diagnostic criteria and therapeutic approaches for pediatric NMOSD.