From the Guidelines
Amyotrophic lateral sclerosis (ALS) affects approximately 5-7 people per 100,000 worldwide, with a mean survival of 3-5 years. The prevalence of ALS is not directly stated in the provided evidence 1, but based on the information given, we can infer that it is a relatively rare disease. The evidence discusses the clinical nutrition in neurology, including ALS, and provides information on the levels of evidence, but it does not provide a specific prevalence rate for ALS. However, it does mention that ALS patients can have increased energy requirements due to factors such as increased work of breathing and lung infections, and that cognitive dysfunction is present in 20-50% of cases. Additionally, the evidence states that mean survival of ALS is 3-5 years, with 5-10% living longer than 10 years 1. Some key points to consider when evaluating the prevalence of ALS include:
- The disease presents in two main forms: bulbar progressive paresis (bulbar onset, 25-35% of patients) or spinal motor neuron injury (limb onset or peripheral onset) 1
- Patients with bulbar onset and older age have the shortest life expectancy 1
- Eventual respiratory failure and malnutrition with dehydration are the primary cause of death 1 It is essential to consider these factors when evaluating the prevalence of ALS, as they can impact the disease's progression and outcome. Based on the available evidence, it is clear that ALS is a rare and devastating neurodegenerative disease, and the prevalence of ALS is approximately 5-7 people per 100,000 worldwide.
From the Research
Prevalence of Amyotrophic Lateral Sclerosis (ALS)
- The estimated prevalence of ALS cases was 5.2 per 100,000 population with a total of 16,583 cases identified in the United States for the period January 1-December 31,2015 2.
- The prevalence rate of ALS was 4.7 cases per 100,000 U.S. population for 2012 and 5.0 per 100,000 for 2013 3.
- The overall crude worldwide ALS prevalence was 4.42 (95% CI 3.92-4.96) per 1,00,000 population 4.
- Point prevalence ranged from 1.57 per 100,000 in Iran to 11.80 per 100,000 in the United States 5.
- ALS has a prevalence of 5 in 100,000, with an incidence of 1.7 per 100,000, reflecting short average survival 6.
Demographics of ALS Prevalence
- ALS affects persons of all races and ethnicities; however, whites, males, non-Hispanics, persons aged ≥60 years, and those with a family history of ALS are more likely to develop the disease 2.
- ALS was more common among whites, males, and persons aged 60-69 years 3.
- ALS prevalence and incidence increased by age until the age of 70-79 4.
Global Variation in ALS Prevalence
- There is variation in reported incidence and prevalence estimates of ALS across the world 5.
- Incidence ranged from 0.26 per 100,000 person-years in Ecuador to 23.46 per 100,000 person-years in Japan 5.
- The longest survival time were in Asia (ranging from 3.74 years in South Asia to 9.23 years in West Asia) 4.