Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset

Mult Scler. 2014 Jul;20(8):1086-94. doi: 10.1177/1352458513515085. Epub 2013 Dec 9.


Background: Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab).

Objective: To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death.

Methods: We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model.

Results: We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate.

Conclusion: LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death.

Keywords: Aging; aquaporin antibody; aquaporin-4; late onset; morbidity; mortality; neuromyelitis optica; prognosis; spectrum disorders.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Aquaporin 4 / immunology
  • Autoantibodies / blood
  • Biomarkers / blood
  • Cause of Death
  • Chi-Square Distribution
  • Disability Evaluation
  • Disease Progression
  • Europe / epidemiology
  • Female
  • Humans
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Motor Activity*
  • Multivariate Analysis
  • Neuromyelitis Optica / diagnosis*
  • Neuromyelitis Optica / immunology
  • Neuromyelitis Optica / mortality
  • Neuromyelitis Optica / physiopathology
  • Predictive Value of Tests
  • Prognosis
  • Proportional Hazards Models
  • Retrospective Studies
  • Risk Factors
  • Severity of Illness Index
  • Time Factors


  • AQP4 protein, human
  • Aquaporin 4
  • Autoantibodies
  • Biomarkers