title: Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.
abstract: 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).
  
  
  
  To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death.
  
  
  
  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.
  
  
  
  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.
  
  
  
  LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death.
authors: Department of Neurology, Hautepierre Hospital, University of Strasbourg, France nicolas.collongues@chru-strasbourg.fr.
authors: N
authors: N
authors: Collongues
authors: Department of Neurology, Pierre Wertheimer Hospital, University of Lyon, France.
authors: R
authors: R
authors: Marignier
authors: The Walton Centre for Neurology and Neurosurgery, The Walton Centre Foundation Trust, Liverpool, UK.
authors: A
authors: A
authors: Jacob
authors: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, UK.
authors: M I
authors: MI
authors: Leite
authors: Department of Neurology, Istanbul University, Turkey.
authors: A
authors: A
authors: Siva
authors: Neurocure, Charité University Medicine Berlin, Germany.
authors: F
authors: F
authors: Paul
authors: Department of Neurology, Robert Salengro Hospital, University of Lille Nord de France, France.
authors: H
authors: H
authors: Zephir
authors: Department of Neurology, Istanbul University, Turkey.
authors: G
authors: G
authors: Akman-Demir
authors: The Walton Centre for Neurology and Neurosurgery, The Walton Centre Foundation Trust, Liverpool, UK.
authors: L
authors: L
authors: Elsone
authors: Division of Molecular Neuroimmunology, University of Heidelberg, Germany.
authors: S
authors: S
authors: Jarius
authors: Department of Neurology, Groupe Hospitalier Pitié Salpétrière, Paris, France.
authors: C
authors: C
authors: Papeix
authors: The Walton Centre for Neurology and Neurosurgery, The Walton Centre Foundation Trust, Liverpool, UK.
authors: K
authors: K
authors: Mutch
authors: Department of Neurology, Istanbul University, Turkey.
authors: S
authors: S
authors: Saip
authors: Division of Molecular Neuroimmunology, University of Heidelberg, Germany.
authors: B
authors: B
authors: Wildemann
authors: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, UK.
authors: J
authors: J
authors: Kitley
authors: Department of Neurology, Hacettepe University, Ankara, Turkey.
authors: R
authors: R
authors: Karabudak
authors: Department of Neurology, Heinrich-Heine-University of Düsseldorf, Germany.
authors: O
authors: O
authors: Aktas
authors: Department of Neurology, Bakýrkoy Hospital for Psychiatric and Neurological Diseases, Istanbul, Turkey.
authors: D
authors: D
authors: Kuscu
authors: Department of Neurology, Istanbul University, Turkey.
authors: A
authors: A
authors: Altintas
authors: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, UK.
authors: J
authors: J
authors: Palace
authors: C
authors: C
authors: Confavreux
authors: Department of Neurology, Hautepierre Hospital, University of Strasbourg, France.
authors: J
authors: J
authors: De Seze
mesh_terms: D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002423:Cause of Death; D016009:Chi-Square Distribution; D004185:Disability Evaluation; D018450:Disease Progression; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009043:Motor Activity; D015999:Multivariate Analysis; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality / Q000503:physiopathology; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D015415:Biomarkers
keywords: Aging; aquaporin antibody; aquaporin-4; late onset; morbidity; mortality; neuromyelitis optica; prognosis; spectrum disorders