Clinical insights and risk factors for disability in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder patients: a multicenter real-world study in Greece.

OBJECTIVE

To characterize the clinical features, disease course and outcomes of aquaporin-4 (AQP4) IgG positive Neuromyelitis Optica Spectrum Disorder (NMOSD) in a Greek cohort.

METHODS

A retrospective analysis of medical records from 93 anti-AQP4 seropositive NMOSD patients, diagnosed according to the 2015 International Panel criteria and followed between 2001-2024 in 16 Neurological Departments, was conducted. Data on demographics, clinical features, disease courses, autoimmune comorbidities, and treatment responses were analyzed.

RESULTS

We identified 93 Caucasian patients (95.7% female), with a mean age at NMOSD onset of 45.6 (±18) years and a mean disease duration of 9.8 (±8.2) years. The most common 1st attack manifestations were transverse myelitis (45.2%) and optic neuritis (32.2%). Autoimmune comorbidities were observed in 32.2% of patients. Age at onset correlated with the clinical phenotype of the 1st attack; myelitis was more frequent in older patients, whereas optic neuritis was more common in younger patients. During follow-up, 55.9% experienced relapses, and the median Expanded Disability Status Scale (EDSS) score was 3. Prophylactic treatment was administered to 89% of patients, with rituximab being the most common therapy. In multivariable logistic regression models older age at disease onset (odds ratio [OR]: 1.069; 95%CI:1.031-1.08, p<0.001), longer disease duration (OR: 1.129; 95%CI:1.022-1.248, p=0.017), and switching between multiple treatments (OR: 2.105; 95%CI:1.029-4.304, p=0.041), were associated with a higher likelihood of severe disability (EDSS ≥4).

CONCLUSIONS

This study highlights an age-related trend in NMOSD presentations and identifies key predictors of disability. Individualized treatment strategies, particularly for older patients, are essential for optimizing disease management.