Brief Report on Juvenile Amyotrophic Lateral Sclerosis Cases in the United States National ALS Registry: 2010 – 2018



Poster Number: M207


Jaime Raymond, MPH, National ALS Registry - CDC/ATSDR, Paul Mehta, MD, National ALS Registry

Background: Amyotrophic lateral sclerosis (ALS) is a progressive, fatal disease with a prevalence rate of approximately 9.9 per 100,000 persons in the United States (U.S.). Published reports suggest 5-10% of ALS cases are classifiable as familial ALS and a much smaller proportion is classified as juvenile ALS (jALS), defined as developing symptom onset before age 25.
Objective: To describe the demographic characteristics of confirmed and likely jALS cases in a large cohort of ALS patients ascertained in the National ALS Registry (Registry).
Methods: Established in 2010, the Registry collects and examines data on ALS patients in the U.S. to better describe the epidemiology of ALS. The Registry compiles data from national administrative databases (i.e., Centers for Medicare and Medicaid Services [CMS], Veterans Health Administration [VHA], Veterans Benefits Administration [VBA]), and from voluntary enrollment data through a web portal. Patients in the Registry must be at least 18 years of age. We examined Registry data from 2010–2018.
Results: The Registry identified 44 patients, aged 18-24, who met the Registry definition of confirmed ALS (30 cases) or likely ALS (14 cases). Of those, 20 (37.8%) were diagnosed at age 24. JALS cases were more likely to be non-White (54.5%), male (79.5%), and live in the Midwest or Northeast regions (54.5%) of the U.S. Some 68.9% of the jALS cases were received from CMS, VHA, or VBA, and 16% came from the web portal only.
Conclusions: Demographic characteristics for jALS cases in the Registry differed from previous publications examining ALS cases for all adults. More research is needed to better understand risk factors contributing to jALS, which could lead to earlier diagnosis and therapeutic interventions.