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.
Statistics from Altmetric.com
▸ 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.
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.