Clinical characteristics of late-onset neuromyelitis optica spectrum disorder

Published:January 15, 2023DOI:


      • Age at onset could have important implications for the long-term prognosis of NMOSD patients.
      • Several age-related disease characteristics, such as a higher proportion of thoracic myelitis, higher AQP4-IgG titters, and more WMHs in LO-NMOSD patients.
      • LO-NMOSD patients had worse outcomes, such as more painful sensory symptoms, sleep disorders, anxiety, and cognitive function, than did those with EO-NMOSD.



      Anti-aquaporin-4 (AQP-4) immunoglobulin G (IgG) is a major autoimmune antibody that contributes to the pathogenesis of neuromyelitis optica spectrum disorder (NMOSD). NMOSD often presents as disability, severe sensory impairment, and sleep disorders, which can cause anxiety and depression and further affect the quality of life. The age of onset is a key factor influencing the prognosis of NMOSD. However, this result was based on studies involving only anti-aquaporin-4 (AQP4) immunoglobulin G (IgG)-seropositive NMOSD patients or studies using the 2006 NMOSD diagnosis criteria. Therefore, further study of the age of onset of NMOSD is valuable. This study aimed to describe the clinical and magnetic resonance imaging (MRI) differences between early-onset neuromyelitis optica spectrum disorder (EO-NMOSD) and late-onset (LO)-NMOSD patients.


      Fifty patients were enrolled, their anti-AQP4-IgG titers were measured, and brain and spinal cord MRIs were obtained. Additionally, several questionnaires related to disease severity, anxiety, depression, cognition, sleep, pain, and fatigue were collected.


      Higher AQP4-IgG seropositivity, higher AQP4-IgG titer, frequency of thoracic myelitis, and white matter hyperintensities (WMH), as well as greater severity of disability, greater severity of sleep disorders, higher anxiety, poorer cognitive function, and higher clinical dementia rating (CDR)-community affairs scores were observed in late-onset (LO)-NMOSD patients than those in early-onset (EO)-NMOSD. AQP4-IgG titer positively correlated with age, annual relapse rate, Expanded Disability Status Scale (EDSS) sensory scores, Activity of Daily Living Scale (ADL) scores, and Pittsburgh Sleep Quality Index (PSQI) scores. The EDSS-sensory scores positively correlated with age, relapse time, Hamilton Depression Rating Scale, Hamilton Anxiety Rating Scale, PSQI, ADL, and CDR. WMH was positively correlated with age, EDSS-sensory scores, PSQI scores, and CDR scores and negatively correlated with the California Verbal Learning Test scores.


      LO-NMOSD patients have worse prognoses than those of EO-NMOSD patients. Higher AQP4-IgG titers, more WMHs, thoracic myelitis, and severe sensory symptoms are associated with cognition, depression, anxiety, and sleep disorders.


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