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

Burchardt, Judith; Mei, Xue W; Ranger, Tom; McDermott, Christopher J.; Radunović, Aleksandar; Coupland, Carol; Hippisley‐Cox, Julia

doi:10.1080/21678421.2021.2016837

Cited by 13 publications

(8 citation statements)

References 41 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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 ).…”

Section: Introductionmentioning

confidence: 99%

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

Cai

et al. 2022

Front. Aging Neurosci.

View full text Add to dashboard Cite

Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by the degeneration and death of motor neurons. Systemic neuroinflammation contributes to the pathogenesis of ALS. The proinflammatory milieu depends on the continuous crosstalk between the peripheral immune system (PIS) and central immune system (CIS). Central nervous system (CNS) resident immune cells interact with the peripheral immune cells via immune substances. Dysfunctional CNS barriers, including the blood–brain barrier, and blood–spinal cord barrier, accelerate the inflammatory process, leading to a systemic self-destructive cycle. This review focuses on the crosstalk between PIS and CIS in ALS. Firstly, we briefly introduce the cellular compartments of CIS and PIS, respectively, and update some new understanding of changes specifically occurring in ALS. Then, we will review previous studies on the alterations of the CNS barriers, and discuss their crucial role in the crosstalk in ALS. Finally, we will review the moveable compartments of the crosstalk, including cytokines, chemokines, and peripheral immune cells which were found to infiltrate the CNS, highlighting the interaction between PIS and CIS. This review aims to provide new insights into pathogenic mechanisms and innovative therapeutic approaches for ALS.

show abstract

Section: Introductionmentioning

confidence: 99%

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

Cai

et al. 2022

Front. Aging Neurosci.

View full text Add to dashboard Cite

show abstract

“…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.…”

Section: Introductionmentioning

confidence: 98%

“…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.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2022

Self Cite

View full text Add to dashboard Cite

ObjectiveTo confirm the symptoms and signs for motor neuron disease (MND) in the Red Flag tool; to quantify the extent to which the key symptoms and signs are associated with MND; and to identify additional factors which may be helpful within the primary care setting in recognition of possible MND and triggering timely referral to neurology specialists.DesignA nested case–control study.Setting1292 UK general practices contributing to the QResearch primary care database, linked to hospital and mortality data.ParticipantsBaseline cohort included 16.8 million individuals aged 18 years and over without a diagnosis of MND at study entry and with more than 3 years of digitalised information available. The nested case–control data set comprised of 6437 cases of MND diagnosed between January 1998 and December 2019, matched by year of birth, gender, general practice and calendar year to 62 003 controls.Main outcome measuresClinically recognised symptoms and signs of MND prior to diagnosis and symptoms and factors which are relevant in primary care setting.ResultsThis study identified 17 signs and symptoms that were independently associated with MND diagnosis in a multivariable analysis. Of these, seven were new to the Red Flag tool: ataxia, dysphasia, weight loss, wheeze, hoarseness of voice, urinary incontinence and constipation. Among those from the Red Flag tool, dysarthria had the strongest association with subsequent MND (adjusted OR (aOR): 43.2 (95% CI 36.0 to 52.0)) followed by muscle fasciculations (aOR: 40.2 (95% CI 25.6 to 63.1)) and muscle wasting (aOR: 31.0 (95% CI 19.5 to 49.4)). Additionally, the associations between MND diagnosis and family history, dropped foot, focal weakness and sialorrhoea remained robust after controlling for confounders. Patients who reported symptoms indicative of damage to the lower brainstem and its connections were diagnosed sooner than those who presented with respiratory or cognitive signs.ConclusionThis is the first study that has identified, confirmed and quantified the association of key symptoms and signs with MND diagnosis. In addition to known factors, the study has identified the following new factors to be independently associated with MND prior to diagnosis: ataxia, dysphasia, wheeze and hoarseness of voice. These findings may be used to improve risk stratification and earlier detection of MND in primary care.

show abstract

“…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.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2023

View full text Add to dashboard Cite

Background: Motor neurone disease is a rare but debilitating illness with incomplete evidence regarding patients’ symptom burden. Palliative care and generalist clinicians are often in-experienced in caring for these patients and assessing their needs. Aim: To identify the symptom prevalence and severity experienced by patients with motor neurone disease. Secondary objectives were to examine differences in symptom burden and clusters according to phenotype, functional status, palliative care provision and those in their last months of life. Design: A point prevalence study assessing patient-reported symptoms using a modified IPOS-Neuro assessment tool, incorporating 41 symptom items. Setting/participants: Patients with motor neurone disease attending the State-wide Progressive Neurological Disease Service or inpatient unit at Calvary Health Care Bethlehem, Melbourne Australia, from March to December 2021. Results: A total of 102 patients participated, the majority diagnosed with lumber-onset (30.4%), bulbar-onset (28.4%) and cervical-onset (25.5%) phenotypes. Patients experienced a median of 17 symptoms (range 2–32) with a median of 3 symptoms rated as severe/overwhelming (range 0–13). Motor and functional symptoms predominated, with differences in symptom clusters present according to phenotype. Patients had a higher number of severe/overwhelming symptoms if they were accessing palliative care services ( p = 0.005), in their last 6 months of life ( p = 0.003) and experiencing moderate or severe functional impairment ( p < 0.001). Conclusions: Patients with motor neurone disease report high symptom burden. A validated motor neurone disease-specific symptom assessment tool is needed to accurately assess patients, including important variations in symptom clusters according to phenotype. Further research must focus on evidence-based treatment guidelines for symptoms experienced commonly and severely.

show abstract

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

Cited by 13 publications

References 41 publications

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

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

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

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

Contact Info

Product

Resources

About