Motor Neuron Disease Register for England, Wales and Northern Ireland—an analysis of incidence in England

Sarah Opie-Martin,Lynn Ossher,Andrea Bredin,Anna Kulka,Neil Pearce,Kevin Talbot,Ammar Al-Chalabi

doi:10.1080/21678421.2020.1812661

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.

Highlights

Amyotrophic lateral sclerosis (ALS) has a reported incidence of 1–2/100,000 person-years
We describe the creation of the MND Register for England, Wales, and Northern Ireland through the incorporation of local population registers, use of data collected routinely to MND register for England 87 organize ALS clinics, and involvement of people with ALS directly through a self-registration website
After data cleaning there were 1748 records, of these, 232 recorded a date of diagnosis between 2017 and 2018, referred to as the complete case dataset. 312 people had no date of diagnosis recorded, but all had a date of onset

Summary

Introduction

Amyotrophic lateral sclerosis (ALS) has a reported incidence of 1–2/100,000 person-years. We describe the establishment of a population register for England, Wales, and Northern Ireland and report-estimated incidence. 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. As the MND Register for England, Wales, and Northern Ireland grows we will update incidence estimates and report on further analyses. Since the initial discovery that mutations in the SOD1 gene can cause ALS, there has been considerable progress in the identification of genetic risk factors [7] Despite these advances, disease etiology in the majority of cases is not understood. Heritability estimates are compatible with the possibility that non-genetic factors such as stochastic biological events in aging, environmental exposures or lifestyle choices contribute to disease risk, but there is no consensus on what these factors are [8]

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Conclusion