The prevalence of neuromyelitis optica in South East Wales

Eur J Neurol. 2012 Apr;19(4):655-9. doi: 10.1111/j.1468-1331.2011.03529.x. Epub 2011 Oct 4.


Background and purpose: Neuromyeltis optica (NMO) is a neuroinflammatory disorder considered rare in Caucasian populations. However, accurate population-based epidemiological data for NMO and NMO spectrum disorder (NMO-SD) from Western populations employing validated diagnostic criteria remain limited. We sought therefore to estimate the prevalence and clinical features of NMO in a north European Caucasian population in South East Wales.

Methods: Patients were identified by a comprehensive, multistage ascertainment strategy employing a regional neuroinflammatory disease register, hospital diagnostic databases personal physician referrals and regional requests for anti-aquaporin-4 antibodies (anti-AQP4).

Results: Fourteen Caucasian patients (11 patients with NMO and three with NMO-SD) were identified in a population of 712,572 (19.6/million; 95% CIs: 12.2-29.7). There was an excess of females (female:male 12:2), 11/14 were anti-AQP4 positive and 5/14 had disease onset under the age of 20 years.

Conclusion: This study suggests that NMO and related spectrum disorders are at least as frequent in Northern European populations as in non-Caucasian populations and that the demographic profile of prevalent patients differs from clinic-based cohorts.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Antibodies / blood
  • Aquaporin 4 / immunology
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Male
  • Middle Aged
  • Neuromyelitis Optica / diagnosis
  • Neuromyelitis Optica / epidemiology*
  • Neuromyelitis Optica / immunology
  • Neuromyelitis Optica / metabolism
  • Prevalence
  • Wales / epidemiology
  • Young Adult


  • AQP4 protein, human
  • Antibodies
  • Aquaporin 4