Global variation in prevalence and incidence of amyotrophic lateral sclerosis: a systematic review and meta-analysis

Xu, Lu; Liu, Tanxin; Liu, Huan; Yao, Xiaoying; Chen, Lu; Fan, Dongsheng; Zhan, Siyan; Wang, Shengfeng

doi:10.1007/s00415-019-09652-y

Cited by 178 publications

(168 citation statements)

References 53 publications

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“…The influence of age group was confirmed by the increase in the prevalence and incidence rates in China after the standardisation of our data to either the European, USA or Australian population 14. However, the incidence and prevalence were still lower than in the corresponding regions even after the standardisation to either the European, USA or Australian population 2 15 16…”

Section: Discussionsupporting

confidence: 63%

Incidence and prevalence of amyotrophic lateral sclerosis in urban China: a national population-based study

Chen

Wang

et al. 2020

J Neurol Neurosurg Psychiatry

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ObjectiveAmyotrophic lateral sclerosis (ALS) is a severe neurodegenerative disease and information on disease burden of ALS in mainland China was limited. We aimed to estimate the prevalence and incidence of ALS in China.MethodsWe used 2012–2016 data from China’s Urban Employee Basic Medical Insurance and Urban Residence Basic Medical Insurance, covering approximately 0.43 billion individuals. ALS cases were identified by the primary diagnosis (International Classification of Diseases code or text of diagnosis) in the insurance database.ResultsThe crude prevalence and incidence in 2016 were 2.91 per 100 000 person-years (95% CI 2.31 to 3.58) and 1.65 (95% CI 1.33 to 2.01), respectively. The standardised prevalence and incidence based on 2010 Chinese census data were 2.97 (95% CI 2.91 to 3.03) and 1.62 (95% CI 1.58 to 1.67), respectively. The annual prevalence between 2013 and 2016 remained relatively constant, ranging from 2.91 (95% CI 2.31 to 3.58) in 2016 to 3.29 (95% CI 2.51 to 4.17) in 2014 (linear regression: β=−0.129, p=0.104). Both rates peaked in the group aged 75–79 years.ConclusionsThe prevalence and incidence of ALS in mainland China were lower than those in developed countries, and maintained a relatively stable trend. The age at onset and age at diagnosis for ALS patients were younger than those in developed countries. Further research is expected to clarify the potential pathophysiological mechanism of ALS.

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Section: Discussionsupporting

confidence: 63%

Incidence and prevalence of amyotrophic lateral sclerosis in urban China: a national population-based study

Chen

Wang

et al. 2020

J Neurol Neurosurg Psychiatry

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“…Amyotrophic lateral sclerosis (ALS) is a highly progressive and fatal motoneuron disease characterized by weakness of muscles, leading to increased muscle wasting, dysarthria, dysphagia and respiratory failure [112][113][114]. The prevalence and incidence in males is higher than females [115]. Extending life expectancy in ALS seems to be dependent on improving our understanding of its pathogenesis mechanisms.…”

Section: Evidence From Epidemiological Studiesmentioning

confidence: 99%

Role of Alcohol Drinking in Alzheimer’s Disease, Parkinson’s Disease, and Amyotrophic Lateral Sclerosis

Peng

Yang²,

Joshi

et al. 2020

IJMS

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Neurodegenerative diseases, including Alzheimer’s disease (AD), Parkinson’s disease (PD) and amyotrophic lateral sclerosis (ALS), increase as the population ages around the world. Environmental factors also play an important role in most cases. Alcohol consumption exists extensively and it acts as one of the environmental factors that promotes these neurodegenerative diseases. The brain is a major target for the actions of alcohol, and heavy alcohol consumption has long been associated with brain damage. Chronic alcohol intake leads to elevated glutamate-induced excitotoxicity, oxidative stress and permanent neuronal damage associated with malnutrition. The relationship and contributing mechanisms of alcohol with these three diseases are different. Epidemiological studies have reported a reduction in the prevalence of Alzheimer’s disease in individuals who drink low amounts of alcohol; low or moderate concentrations of ethanol protect against β-amyloid (Aβ) toxicity in hippocampal neurons; and excessive amounts of ethanol increase accumulation of Aβ and Tau phosphorylation. Alcohol has been suggested to be either protective of, or not associated with, PD. However, experimental animal studies indicate that chronic heavy alcohol consumption may have dopamine neurotoxic effects through the induction of Cytochrome P450 2E1 (CYP2E1) and an increase in the amount of α-Synuclein (αSYN) relevant to PD. The findings on the association between alcohol consumption and ALS are inconsistent; a recent population-based study suggests that alcohol drinking seems to not influence the risk of developing ALS. Additional research is needed to clarify the potential etiological involvement of alcohol intake in causing or resulting in major neurodegenerative diseases, which will eventually lead to potential therapeutics against these alcoholic neurodegenerative diseases.

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“…The disease mainly manifests as progressive muscle atrophy, weakness, and respiratory failure 1,2 . According to a systematic review of 2019, the global ALS prevalence and incidence were 4.42/100 000 and 1.59/100 000/year respectively 3 . Despite numerous research efforts over the past decades, the mechanisms of ALS pathogenesis remain unclear.…”

Section: Introductionmentioning

confidence: 99%

UCHL1 from serum and CSF is a candidate biomarker for amyotrophic lateral sclerosis

Wang

Xie

et al. 2020

Ann Clin Transl Neurol

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Objective To identify potential ALS biomarkers in patients and to evaluate their diagnostic performance using cerebrospinal fluid (CSF) and serum. Method We recruited a discovery cohort, comprising 20 ALS patients and 20 controls to screen for potential CSF biomarker, UCHL1, using a Luminex neurodegenerative disease panel. To validate UCHL1’s diagnostic performance, we used receiver operating characteristic (ROC) curves to determine the potential for early diagnosis in another cohort comprising 23 CSF and 69 serum ALS samples. Finally, we analyzed its correlation with clinical features. Results We found significantly elevated levels of CSF‐derived UCHL1 in both discovery and validation cohorts (P < 0.05). ROC curves revealed an AUC of 0.8288, with a sensitivity and specificity of 73.91% and 81.25%, respectively, when the cut‐off value for UCHL1 was >291.9 pg/mL. A similar result was observed in the serum cohort, with the ALS group exhibiting significantly higher serum UCHL1 levels than the controls (P < 0.05). AUC of the ROC in the serum UCHL1 cohort was 0.7709, with sensitivity and specificity of 61.43% and 79.59%, respectively, when the cut‐off value of serum UCHL1 was >15.22 pg/mL. At the early stage CSF and serum UCHL1 were significantly different between ALS patients and controls (P < 0.05). Furthermore, serum UCHL1 levels showed a positive relationship with the burden of UMN and LMN dysfunction, albeit with no statistical significance. Interpretation Taken together, our findings suggest that ALS patients exhibit significantly elevated CSF‐ and serum‐derived UCHL1. Moreover, our data warrant that UCHL1 displays good diagnostic performance and provide novel options for ALS early diagnosis. However, its prognostic value needs to be further investigated.

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Global variation in prevalence and incidence of amyotrophic lateral sclerosis: a systematic review and meta-analysis

Cited by 178 publications

References 53 publications

Incidence and prevalence of amyotrophic lateral sclerosis in urban China: a national population-based study

Incidence and prevalence of amyotrophic lateral sclerosis in urban China: a national population-based study

Role of Alcohol Drinking in Alzheimer’s Disease, Parkinson’s Disease, and Amyotrophic Lateral Sclerosis

UCHL1 from serum and CSF is a candidate biomarker for amyotrophic lateral sclerosis

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