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

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Abstract

Background

Amyotrophic lateral sclerosis (ALS) is a global disease, which adversely affects the life quality of patients and significantly increases the burden of families and society. We aimed to assess the changing incidence, prevalence of ALS around the world.

Methods

We searched Medline, Embase, Web of Science, and Cochrane library to identify articles published until September 9, 2018. Each included study was independently reviewed for methodological quality by two reviewers. We used a random-effects model to summarize individual studies and assessed heterogeneity (I2) with the χ2 test on Cochrane’s Q statistic.

Results

We identified 124 studies that were eligible for final inclusion, including 110 studies of incidence and 58 studies of prevalence. The overall crude worldwide ALS prevalence and incidence were 4.42 (95% CI 3.92–4.96) per 1,00,000 population and 1.59 (95% CI 1.39–1.81) per 1,00,000 person-years, respectively. ALS prevalence and incidence increased by age until the age of 70–79. Since 1957, incidence has been significantly rising year by year, and this upward trend was weakened after standardization. The longest survival time were in Asia (ranging from 3.74 years in South Asia to 9.23 years in West Asia).

Conclusions

With the aggravation of population aging and the rapid growth of economy, developing regions following the development pattern of the developed regions may suffer rising ALS prevalence and incidence which may increase their disease burden as well. These data highlight the need for research into underlying mechanism and innovations in health-care systems.

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Acknowledgements

The authors would like to thank Xuefeng Lai, Jinlang Lv, Junyou Yu, Wei Zhang, and Huan Yu for retrieving and managing articles as well as extracting data.

Funding

This study was funded by the National Natural Science Foundation (grant number 91646107).

The manuscript does not contain clinical studies or patient data.

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Authors and Affiliations

  1. Department of Epidemiology and Biostatistics, School of Public Health, Peking University Health Science Center, Beijing, China

    Lu Xu, Tanxin Liu, Lili Liu, Xiaoying Yao, Siyan Zhan & Shengfeng Wang

  2. Department of Neurology, Peking University Third Hospital, Beijing, China

    Lu Chen & Dongsheng Fan

  3. Center for Clinical Epidemiology, Peking University Third Hospital, Beijing, China

    Siyan Zhan

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  1. Lu Xu
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Xu, L., Liu, T., Liu, L. et al. Global variation in prevalence and incidence of amyotrophic lateral sclerosis: a systematic review and meta-analysis. J Neurol 267, 944–953 (2020). https://doi.org/10.1007/s00415-019-09652-y

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