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Publications (2)8.56 Total impact

  • Article: The prevalence of neuromyelitis optica in South East Wales.
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    ABSTRACT: 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. 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). 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. 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.
    European Journal of Neurology 10/2011; 19(4):655-9. · 3.69 Impact Factor
  • Article: PATU10 Neuromyelitis optica and related spectrum disorders in a UK population-based sample.
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    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.
    Journal of neurology, neurosurgery, and psychiatry 11/2010; 81(11):e26-7. · 4.87 Impact Factor