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Relapsing neuromyelitis optica: long term history and clinical predictors of death
  1. P Cabre1,
  2. A González-Quevedo2,
  3. M Bonnan1,
  4. A Saiz3,
  5. S Olindo1,
  6. F Graus3,
  7. D Smadja1,
  8. H Merle4,
  9. L Thomas5,
  10. J A Cabrera-Gomez6
  1. 1
    Department of Neurology, Pierre Zobda Quitman Hospital, Fort de France, Martinique, French West Indies
  2. 2
    Cuban Multiple Sclerosis Society, Institute of Neurology and Neurosurgery, Havana, Cuba and National Institute of Neurology and Neurosurgery, Havana, Cuba
  3. 3
    Service of Neurology Hospital Clinic and Institut d’Invesigación Biomèdica August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
  4. 4
    Department of Ophthalmology, Pierre Zobda Quitman Hospital, Fort de France, Martinique, French West Indies
  5. 5
    Emergency Department, Pierre Zobda Quitman Hospital, Fort de France, Martinique, French West Indies
  6. 6
    Cuban Multiple Sclerosis Society and Multiple Sclerosis Clinic, Internacional Neurological Centre, Habana, Cuba and International Centre of Neurological Restoration (CIREN), Habana, Cuba
  1. Correspondence to P Cabre, Department of Neurology, Pierre Zobda Quitman Hospital, Fort de France, 97261 Martinique, French West Indies; Philippe.Cabre{at}chu-fortdefrance.fr

Abstract

Background: Relapsing neuromyelitis optica (RNMO) is an uncommon but devastating inflammatory disorder of the central nervous system. Long term history in a wide series of RNMO is required for better knowledge of the course of the disease and identification of patients at high risk of death.

Methods: Clinical features of patients with RNMO (88 women/eight men) obtained from the geographic Caribbean database (Cuba and French West Indies) were used to determine the progression of disability and to identify clinical predictors of death.

Results: Median age at onset of RNMO was 29.5 years (range 11–74). Median duration of disease was 9.5 years (1–40). Median relapse rate was 0.7 attack/patient/year (0.1–3). 66 patients experienced severe visual loss in at least one eye and 46 in both eyes. Median time from onset to unilateral and bilateral severe visual loss was 3 and 15 years, respectively. Median times to reach Kurtzke Disability Status Scale 3, 6 and 8 from onset of RNMO were 1, 8 and 22 years. There were 24 deaths (25%); within 5 years in 63% of cases. A higher attack frequency during the first year of disease (p = 0.009), blindness (p = 0.04) and sphincter signs at onset (p = 0.02) and lack of recovery of first attack (p = 0.003) were independently associated with a shorter time to death.

Conclusion: RNMO is a very rapidly disabling disease affecting primarily young women. This study has identified clinical features that predict a poor outcome. These findings suggest that early and aggressive immunotherapy might be warranted in RNMO.

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Footnotes

  • ▸ Supplementary data are published online only at http://jnnp.bmj.com/content/vol80/issue10

  • Funding NMO-IgG antibody was assessed by a grant support, in part from Fondo de Investigaciones Sanitarios, Madrid, Spain (PI060070; AS, FG) and U344 Inserm unit, Lyon, France (Romain Marignier MD).

  • Competing interests None.

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