The prevalence of neuromyelitis optica in South East Wales

The prevalence of neuromyelitis optica in South East Wales
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DOI:
10.1111/j.1468-1331.2011.03529.x
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发表时间:
2012-04-01
影响因子:
5.1
通讯作者:
Robertson, N.
Robertson, N.
中科院分区:
医学3区
文献类型:
--
作者:
Cossburn, M.;Tackley, G.;Robertson, N.

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背景和目的:视神经脊髓炎(NMO)是一种神经炎症性疾病,在高加索人群中被认为罕见。然而,来自西方人群的 NMO 和 NMO 谱系障碍 (NMO-SD) 采用经过验证的诊断标准的准确的基于人群的流行病学数据仍然有限。因此,我们试图估计威尔士东南部北欧高加索人群中 NMO 的患病率和临床特征。方法:通过采用区域神经炎症疾病登记、医院诊断数据库、个人医生转诊和抗水通道蛋白 4 抗体(抗 AQP4)区域请求的综合、多阶段确定策略来识别患者。结果:在 712 名人群中确定了 14 名白人患者(11 名 NMO 患者和 3 名 NMO-SD 患者) 572(19.6/百万;95% CI:12.2-29.7)。女性较多(女性:男性 12:2),11/14 的人抗 AQP4 阳性,5/14 的人在 20 岁以下发病。 结论:这项研究表明,NMO 和相关谱系疾病在北欧人群中的发病率至少与非白种人人群中的频率相同,而且流行患者的人口统计学特征与临床队列不同。
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.