Disease Characteristics of Seropositive Neuromyelitis Optica Spectrum Disorder in a Turkish Cohort

Abstract

Objective To determine the clinical, demographic and imaging characteristics of a Turkish cohort with aquaporin-4-antibody positive (AQP4-IgG+) neuromyelitis optica spectrum disorder (NMOSD) from a single center. Background NA. Design/Methods 35 patients seen between January-2008 and December-2020 with a diagnosis of AQP4-IgG+NMOSD who could be studied in detail were included in the study. Inclusion criteria for patients with NMOSD diagnosis was defined according to International Consensus Diagnostic Criteria (Wingerchuk et al.2015) and all patients were confirmed for AQP4-IgG positive serology at least once by Euroimmune transfected cells assay (EU90). Demographic, clinical and MRI data were obtained retrospectively. Results The female-to-male ratio was 16.5: 1. The mean age of disease onset was 26,16±10,96 years for patients with optic neuritis onset (n:12), and 43.17±11,95 for the subgroup that started with transverse myelitis (TM) (n:16), confirming a significant difference of age at onset according to the first attack type (p < 0.001). The mean age at onset in 5 patients with area postrema syndrome was 35,74±16,83. Half of the total attacks occurred within the first year of disease onset (98/196). The mean time to diagnosis was 2,98±5,78 years after the initial attack. Disease duration was 10,06±9,76 years. Cerebrospinal fluid oligoclonal bands were studied in 24 and were positive in 25%. An autoimmune rheumatologic disease comorbidity was present in 34.5% of the patients. In patients with MRI disclosing = 2 McDonald dissemination in space criteria (spinal included) was more common in TM group and correlated with a higher disability (EDSS) score. Conclusions Turkish AQP4-IgG+NMOSD patients whose disease start with optic neuritis have an earlier age of onset compared to the ones with TM onset. Half of the total attacks occur within the first year of disease onset. Patients with = 2 McDonald MRI dissemination in space criteria were more common in the TM group and had a higher disability (EDSS) score.