Article Text

PDF
PATU10 Neuromyelitis optica and related spectrum disorders in a UK population-based sample
  1. G Tackley,
  2. M T Burtonwood,
  3. G Ingram,
  4. M D Cossburn,
  5. G A Malik,
  6. T Pickersgill,
  7. N P Robertson
  1. University Hospital of Wales, Cardiff University, Cardiff, UK
  1. Correspondence to tackley{at}gmail.com

Abstract

Neuromyelitis Optica (NMO) is a rare neuroinflammatory disorder with limited epidemiological data. Antibodies against aquaporin-4 (Aq4ab) are reported to be highly specific for NMO and NMO spectrum disorders (NMO-SD). In this study we determine the prevalence of these disorders and spectrum of clinical phenotype in a population-based sample and analyse clinical features that predict Aq4ab positivity. Cases were identified from the SE Wales neuroinflammatory database and regional requests for Aquaporin-4 testing. 10 patients (M2:8, age range 7–70) with NMO were identified of which 7 had positive Aq4ab, as well as two female patients with Aq4ab positive NMO-SD; a combined prevalence of 16/million (95% CI 10 to 25). Median disease duration was 4 years (range 1–34) and median relapse frequency 0.63/year (0.14–1.60). Median MS severity score (MSSS) was 8.10 (4.35–9.59) One third of cases had severe visual impairment. MSSS did not differ between those treated with immunosuppressants (5/12) and those not. Both NMO-SD cases had isolated longitudinally extensive transverse myelitis (LETM). Aq-4ab were positive in 9/79 (11.4%) sample requests. Test requests were most commonly associated with poorly recovered optic neuritis (56.6%). Logistic regression identified the presence of LETM on MR as the most predictive feature for NMO (Likelihood ratio=5.43). In this study we have established disease prevalence for NMO in a UK population-based sample and identified LETM as the main indication for Aq4ab testing.

Statistics from Altmetric.com

Request permissions

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.