Analysis of incidence of motor neuron disease in England 1998–2019: use of three linked datasets

Figures & data

Table 1 Incidence rates of MND from Qresearch compared to previous studies of MND in Great Britain.

Study	Setting	Study period	N incidence	Sample size	Age	Crude incidence rate /100 000 Person Age Years (95% confidence intervals)	Age-standardized to UK 2011 population all ages incidence rate /100 000 person years (95% confidence intervals)	Age-standardized to US 2010 population all ages incidence rate /100,000 person years (95% confidence intervals)
QResearch (this study)	England (GP, HES, or death)	1998–2019	6437	16,799,922 (112,003,453 patient years)	18+	5.75 (5.61–5.89)	5.69 (5.51–5.88)	5.02 (4.95–5.10)
Opie-Martin et al. (2020) (11)	England (Six MND registers including imputed cases)	2017–2018	287–301	7,251,845	16+	–	2.072 (2.072–2.073)	1.874 (1.873–1.874)
Leighton et al (2019) (5)	Scottish MND Register	2015–2016	406	5,388,850	16+	3.83 (3.53–4.14)	–	3.42 (2.99–3.9) in 2015 2.89 (2.50–3.34) in 2016
Gowland et al (2019) (12)	South London and Canterbury (SEALS register)	2004–2017	415	–	15+	2.27 (2.04–2.50)	2.74 (2.59–2.88)^b	–
Imam et al (2010) (9)	Devon and Cornwall, England (hospital register)	2002–2007	243	–	15+	2.98	–	–
Alonso et al (2009) (7)	England^a (GP data from CPRD database)	1990–2005	830	>3,000,000 (22,492,571 person years)	20+	3.69 (3.44–3.95)	–	–
Abhinav et al (2007) (6)	South East England (ALS Register)	2002–2006	138	2,890,482	15+	1.06	–	–
Forbes et al. (2007) (16)	Scottish MND Register	1989–1998	1226	5,1215,000	10+	2.38 (2.25–2.52)	–	–
Mitchell et al. (1998) (10)	Lancashire and South Cumbria, England (neurologist knowledge)	1989–1993	130	1,473,153	NS	1.76	–	–

^aAlthough this paper is reported as being from the UK, the CPRD (then GPRD) database from which it was taken was only linked to English practices at the time. Crude rate and confidence intervals were calculated from data given in the paper.

^b2010 UK population. NS: not specified.

Table 2 Crude and age-standardized incidence of MND (95% confidence intervals) and median age by data source.

	All MND patients (including death register) (n = 6437)	Male MND patients (including death register) (n = 3656)	Female MND patients (including death register) (n = 2781)
Crude incidence adult MND/100,000 adult years	5.75 (5.61–5.89)	6.61 (6.40–6.83)	4.91 (4.72–5.09)
UK 2011 age-standardized incidence/100,000 person years using all 3 datasets	5.69 (5.51–5.88)	6.69 (6.41–6.99)	4.72 (4.49–4.97)
US 2010 age-standardized Incidence/100,000 person years using all 3 datasets	5.02 (4.95–5.10)	5.82 (5.70–5.94)	4.26 (4.16–4.36)
Median age of diagnosis in years (interquartile range)	72 (64–80)	72 (63–79)	73 (65–80)
	MND patients in HES and QResearch (n = 5773)	Male MND patients in HES and QResearch (n = 3271)	Female MND patients in HES and QResearch (n = 2502)
UK 2011 age-standardized Incidence/100,000 person years excluding death registry patients	5.23 (5.05–5.41)	6.01 (5.74–6.29)	4.47 (4.24–4.71)
Median age of diagnosis in years (interquartile range)	72 (63–79)	71 (63–78)	73 (65–80)

Figure 1 Data sources for MND cases – GP records, Hospital Episode Statistics and Death Certificates 1998–2019.

Figure 2 Crude MND Incidence/100,000 adult years in England 1998–2019.

Figure 3 Crude incidence by age at study entry for MND/100,000 adult years by age and sex in England 1998–2019.

Figure 4 Age-standardized incidence MND/100,000 person years in England by self-reported ethnicity 1998–2019.

Opie-Martin S, Ossher L, Bredin A, Kulka A, Pearce N, Talbot K, et al. Motor Neuron Disease Register for England, Wales and Northern Ireland—an analysis of incidence in England. Amyotroph Lateral Scler Frontotemporal Degener. 2021;22:86–8.

 PubMed Web of Science ®Google Scholar

Leighton DJ, Newton J, Stephenson LJ, Colville S, Davenport R, Gorrie G, CARE-MND Consortium, et al. Changing epidemiology of motor neurone disease in Scotland. J Neurol. 2019;266:817–25.

 PubMed Web of Science ®Google Scholar

Gowland A, Opie-Martin S, Scott KM, Jones AR, Mehta PR, Batts CJ, et al. Predicting the future of ALS: the impact of demographic change and potential new treatments on the prevalence of ALS in the United Kingdom, 2020–2116. Amyotroph Lateral Scler Frontotemporal Degener. 2019;20:264–74.

 PubMed Web of Science ®Google Scholar

Imam I, Ball S, Wright D, Hanemann CO, Zajicek J. The epidemiology of motor neurone disease in two counties in the southwest of England. J Neurol. 2010;257:977–81.

 PubMed Web of Science ®Google Scholar

Alonso A, Logroscino G, Jick SS, Hernan MA. Incidence and lifetime risk of motor neuron disease in the United Kingdom: a population-based study. Eur J Neurol. 2009;16:745–51.

 PubMed Web of Science ®Google Scholar

Abhinav K, Stanton B, Johnston C, Hardstaff J, Orrell RW, Howard R, et al. Amyotrophic lateral sclerosis in South-East England: a population-based study. The South-East England register for amyotrophic lateral sclerosis (SEALS Registry). Neuroepidemiology. 2007;29:44–8.

 PubMed Web of Science ®Google Scholar

Forbes RB, Colville S, Parratt J, Swingler RJ. The incidence of motor nueron disease in Scotland. J Neurol. 2007;254:866–9.

 PubMed Web of Science ®Google Scholar

Mitchell JD, Gatrell AC, Al-Hamad A, Davies RB, Batterby G. Geographical epidemiology of residence of patients with motor neuron disease in Lancashire and south Cumbria. J Neurol Neurosurg Psychiatry. 1998;65:842–7.

 PubMed Web of Science ®Google Scholar

Data availability statement

The study protocol and statistical analysis plan for this project are available on request from the corresponding author. De-identified individual participant data that underlie the results reported in this article will be made available, with requests accepted immediately after publication, for proposals that set out to achieve aims specified in a methodologically and scientifically sound protocol that are approved by the Qresearch Scientific Advisory Committee (“learned intermediary“), where costs of providing access to the data are covered, where requests are compliant with the legal permissions of Qresearch data providers, and Qresearch data security requirements are met. Information regarding submission of applications to access data can be found online: www.qresearch.org/.

Figure 1 Data sources for MND cases – GP records, Hospital Episode Statistics and Death Certificates 1998–2019.

Figure 2 Crude MND Incidence/100,000 adult years in England 1998–2019.

Figure 3 Crude incidence by age at study entry for MND/100,000 adult years by age and sex in England 1998–2019.

Figure 4 Age-standardized incidence MND/100,000 person years in England by self-reported ethnicity 1998–2019.