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

Abstract

Objective: This study uses three linked datasets to provide an estimate of incidence of motor neuron disease (MND) in England from 1998 to 2019. Comparison is made to previous British studies. It examines age at diagnosis and ethnicity of those affected. Methods: The literature was searched for studies of MND incidence in Great Britain from 1995 to date. The QResearch and linked Hospital Episode Statistics and Death register databases were searched from 1998 to 2019 for cases of MND, and incidence calculated from 16.8 million adults and 112 million adult years of data. Results: We found 6437 adults with a diagnosis of MND giving an incidence of MND of 5.69/100,000 person years (95% CI 5.51–5.88); 6.57 (6.41–6.99) in men and 4.72 (4.49–4.97) in women when age-standardized to the 2011 UK population. The median age of diagnosis was 72 years. Peak incidence occurred in the 80–84 year age group in men and 75–79 in women. Age-standardized incidence was as high in Bangladeshi, Black Caribbean, Indian, other Asian and Pakistani people as in White people. Black African and Chinese people had a lower incidence. Conclusion: The use of three linked national datasets captured 33% more people than a primary care dataset alone. Patients were older than in previous studies and rates were high in all ethnic groups studied except Black African and Chinese people. We present the highest incidence of MND reported globally in the past 50 years. Methodological differences may in part explain differences with previous reports. The use of national datasets may have captured additional MND patients with serious comorbidities who have not seen a neurologist before death. A limitation of this approach is that unlike population registers, which minimize false positive diagnosis by neurologist review of each patient, we cannot review diagnosis for individuals as data are anonymized.

Neuroimmune Crosstalk Between the Peripheral and the Central Immune System in Amyotrophic Lateral Sclerosis

“…Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease typically characterized by adult-onset dysfunction of both upper and lower motor neurons (MNs). The incidence rates of this fatal disease were 1.38 (urban China), 1.5 (United States), 2.08 (Europe) per 100,000 persons ( Xu et al, 2020 ; Burchardt et al, 2022 ; Mehta et al, 2022 ), and most patients died within 3–5 years after disease onset ( Chia et al, 2018 ). No clinical therapies have been proven effective except for riluzole and edaravone, which can only delay disease progression ( Chen et al, 2016 ; Scott, 2017 ; Shefner et al, 2020 ).…”

Identifying key signs of motor neurone disease in primary care: a nested case–control study using the QResearch database

“…Motor neuron disease (MND) causes progressive neuromuscular weakness that may first present as isolated and unexplained symptoms. MND causes the deaths of 1 in 350 men and 1 in 470 women in the UK,1 although there is emerging evidence that the incidence might be higher than previously thought 2. In Europe, the annual crude incidence rate was estimated to be 2.7 per 100 000 person-years 3.…”

“…MND causes the deaths of 1 in 350 men and 1 in 470 women in the UK, 1 although there is emerging evidence that the incidence might be higher than previously thought. 2 In Europe, the annual crude incidence rate was estimated to be 2.7 per 100 000 person-years. 3 MND is difficult to recognise in primary care since it is both a relatively uncommon disease and the clinical presentations of early symptoms are sporadic and non-specific.…”

Motor neurone disease: A point-prevalence study of patient reported symptom prevalence, severity and palliative care needs

“…2 Compared to other chronic illnesses, globally motor neurone disease is relatively rare. 1,[12][13][14] A comprehensive systematic review of the literature published in 2014, comparing symptoms and prevalence of palliative carerelated problems in people living with cancer and eight non-cancer conditions, showed symptom prevalence data was the most deficient for patients with motor neurone disease. 15 Specifically, this study identified no data available regarding the prevalence of fatigue, anorexia, nausea/ vomiting, confusion/cognitive impairment, diarrhoea, social or spiritual needs for patients with motor neurone disease.…”