Myelitis-Predominant Aggressive Phenotype: Unveiling Unique Patterns of Late-Onset Neuromyelitis Optica Spectrum...

Area of Science:

• Neurology
• Immunology
• Neuroinflammation

Background:

• Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease affecting the central nervous system.
• Late-onset NMOSD (LO-NMOSD), with onset at age 60 or later, presents unique clinical challenges compared to adult-onset NMOSD (AO-NMOSD).

Purpose of the Study:

• To compare the clinical features and prognosis of LO-NMOSD with AO-NMOSD.
• To offer insights for the individualized management of elderly NMOSD patients.

Main Methods:

• Analysis of data from 748 NMOSD patients in the China National Registry of Neuro-Inflammatory Diseases (CNRID).
• Stratification of patients into AO-NMOSD (18-59 years) and LO-NMOSD (≥60 years).
• Comparison of demographics, clinical data, imaging, treatments, and outcomes using Kaplan-Meier curves and Cox regression analysis.

Main Results:

• LO-NMOSD exhibited lower female predominance, higher incidence of transverse myelitis (TM) at onset, and increased annualized relapse rate (ARR).
• Patients with LO-NMOSD experienced accelerated disability progression (median EDSS 4.75 vs 3.0) and a significantly higher risk of relapse, disability, and severe visual impairment.
• TM-predominant relapses were a major contributor to disability in LO-NMOSD.

Conclusions:

• LO-NMOSD is characterized by myelitis predominance, recurrent spinal cord involvement, and a high risk of relapses and severe disability.
• Elderly patients with NMOSD require heightened clinical attention and rigorous monitoring.
• Management strategies for LO-NMOSD should balance safety and efficacy, considering the distinct disease characteristics in older adults.