Abstract

Introduction

Amyotrophic lateral sclerosis (ALS) has a reported incidence of 1–2/100,000 person-years. It is estimated that there are 5000 people with ALS in the UK at any one time; however, the true figure and geographical distribution, are unknown. In this study, we describe the establishment of a population register for England, Wales, and Northern Ireland and report-estimated incidence. Methods: People with a diagnosis of ALS given by a consultant neurologist and whose postcode of residence is within England, Wales, or Northern Ireland were eligible. The catchment area was based on six data contributors that had been participating since 2016. All centres included in this analysis were in England, and therefore Wales and Northern Ireland are not included in this report. Crude age- and sex-specific incidence rates were estimated using population census records for the relevant postcodes from Office of National Statistics census data. These rates were standardized to the UK population structure using direct standardization. Results: There were 232 people in the database with a date of diagnosis between 2017 and 2018, when missing data were imputed there were an estimated 287–301 people. The denominator population of the catchment area is 7,251,845 according to 2011 UK census data. Age- and sex-adjusted incidence for complete cases was 1.61/100,000 person-years (95% confidence interval 1.58, 1.63), and for imputed datasets was 2.072/100,000 person-years (95% CI 2.072, 2.073). Discussion: We found incidence in this previously unreported area of the UK to be similar to other published estimates. As the MND Register for England, Wales, and Northern Ireland grows we will update incidence estimates and report on further analyses.

中文翻译：

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英格兰、威尔士和北爱尔兰运动神经元疾病登记——英格兰发病率分析

摘要

介绍

据报道，肌萎缩侧索硬化症 (ALS) 的发病率为 1-2/100,000 人年。据估计，英国任何时候都有 5000 人患有 ALS；然而，真实的数字和地理分布，是未知的。在这项研究中，我们描述了英格兰、威尔士和北爱尔兰人口登记册的建立，并报告了估计的发病率。方法：由顾问神经科医生诊断为 ALS 且居住邮政编码在英格兰、威尔士或北爱尔兰境内的人符合条件。集水区基于自 2016 年以来一直参与的六个数据贡献者。本分析中包含的所有中心均位于英格兰，因此本报告不包括威尔士和北爱尔兰。使用来自国家统计局人口普查数据的相关邮政编码的人口普查记录估计了粗略的年龄和性别特定发病率。使用直接标准化将这些比率标准化为英国人口结构。结果：数据库中有 232 人的诊断日期在 2017 年至 2018 年之间，当估算缺失数据时，估计有 287-301 人。根据 2011 年英国人口普查数据，该集水区的分母人口为 7,251,845。完整病例的年龄和性别调整发病率为 1.61/100,000 人年（95% 置信区间 1.58, 1.63），估算数据集为 2.072/100,000 人年（95% CI 2.072, 2.073）。讨论：我们发现英国这个以前未报告的地区的发病率与其他已发表的估计值相似。随着英格兰、威尔士和北爱尔兰 MND 登记册的增长，我们将更新发病率估计并报告进一步的分析。