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

  • Lu Xu
  • Tanxin Liu
  • Lili Liu
  • Xiaoying Yao
  • Lu Chen
  • Dongsheng Fan
  • Siyan ZhanEmail author
  • Shengfeng WangEmail author
Original Communication



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.


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.


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).


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.


Amyotrophic lateral sclerosis Incidence Prevalence Meta-analysis 



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.


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

The manuscript does not contain clinical studies or patient data.

Compliance with ethical standards

Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Supplementary material

415_2019_9652_MOESM1_ESM.pdf (1.8 mb)
Supplementary file1 (PDF 1797 kb)


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Authors and Affiliations

  1. 1.Department of Epidemiology and Biostatistics, School of Public HealthPeking University Health Science CenterBeijingChina
  2. 2.Department of NeurologyPeking University Third HospitalBeijingChina
  3. 3.Center for Clinical EpidemiologyPeking University Third HospitalBeijingChina

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