Prevalence of amyotrophic lateral sclerosis (ALS), United States, 2016

Abstract

Objective: To estimate the prevalence of amyotrophic lateral sclerosis (ALS) in the United States for 2016 using data from the National ALS Registry (Registry). Established in 2009, the Registry collects data on ALS patients in the U.S. to better describe the epidemiology of ALS, examine risk factors such as environmental and occupational exposures, and characterize the demographics of those living with the disease. Methods: To identify adult prevalent cases of ALS, the Registry compiles data from three national administrative databases (maintained by the Centers for Medicare and Medicaid Services, the Veterans Health Administration, and the Veterans Benefits Administration). To ascertain cases not necessarily included in these databases and to better understand risk-factors associated with ALS and disease progression, the Registry also includes data collected from patients who voluntarily enroll via a web portal to complete online surveys. Results: In 2016, the Registry conservatively identified 16,424 adult persons who met the Registry definition of ALS for an age-adjusted prevalence rate of 5.2 per 100,000 U.S. population. The pattern of patient characteristics (e.g., age, sex, and race/ethnicity) has not changed from previous Registry reports. Overall, ALS was more common among whites, males, and persons aged 60-69 years. The age groups with the lowest number of ALS cases were persons aged 18-39 years. Males had a higher prevalence rate of ALS than females overall and across all data sources. Conclusions: Data collected by the National ALS Registry are being used to better describe the epidemiology and demographics of ALS in the U.S.

Intravenous edaravone treatment in ALS and survival: An exploratory, retrospective, administrative claims analysis

“… 1 The prevalence rate in White people is 1.5 times that in Black people, although the disease course is comparable in both groups, with a median survival of 23.3 months from diagnosis in White people and 25.3 months from diagnosis in Black people. 1 , 2 , 3 The clinical trajectories and survival experiences are similar in population- and clinic-based observational studies. 2 , 3 , 4 …”

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterised by motor neuron cell death and progressive muscular weakness that leads to paralysis. 1 , 2 The estimated prevalence rate for 2016 was 5.2 per 100,000 population. ALS prevalence is lowest (0.2 per 100,000 population) in the 18-to-39 age group and highest (17.2 per 100,000 population) in the 70-to-79 age group.…”

Modeling of SGLT1 in Reconstituted Systems Reveals Apparent Ion-Dependencies of Glucose Uptake and Strengthens the Notion of Water-Permeable Apo States

“…Solute carriers (SLCs) serve as gatekeepers for vital physiological functions, including cellular uptake of minerals, vitamins, and nutrients as well as removal of metabolites from the interior of cells ( Hediger et al, 2004 ; Omote et al, 2006 ; Sano et al, 2020 ). Mutations in the genes encoding SLC proteins are linked to a plethora of diseases, such as amyotrophic lateral sclerosis [prevalence in the United States in 2016; 0.52% ( Mehta et al, 2021 )], Alzheimer’s disease [prevalence in Europe; 5.05% ( Niu et al, 2017 )], and schizophrenia [prevalence in 46 countries worldwide; 0.46% ( Saha et al, 2005 )]. Extrapolated to the world population of 2021, these three conditions alone amount to as many as 470 million annual patients.…”

Effects of PB‐TURSO on the transcriptional and metabolic landscape of sporadic ALS fibroblasts

“…ALS is a progressive and fatal neurodegenerative disorder that affects approximately every 5 per 100,000 people in the United States. 1 The disease involves degeneration of both upper and lower motor neurons, causing muscle weakness and atrophy, spasticity, dysphagia, and neurocognitive symptoms. Eventual paralysis and death due to respiratory insufficiency typically occur within 2-5 years of diagnosis.…”