Determining the Feasibility of a National Amyotrophic Lateral Sclerosis Registry through Four Pilot Projects

Amyotrophic Lateral Sclerosis (ALS) is a progressive and often fatal disease of the upper and lower motor neurons. Most people die within 2–5 years of being diagnosed with ALS. In 2008, the President signed into law the ALS Registry Act, allowing the Agency for Toxic Substances and Disease Registry (ATSDR) to create a national ALS registry to better describe the incidence, prevalence, and potential risk factors of ALS. While the National ALS Registry was officially launched in October, 2010, four pilot projects were conducted prior to the launch to determine the feasibility of various methods to capture ALS cases. The four pilot sites utilized national administrative databases (i.e., Medicare, Medicaid, Veterans Health Administration and Veterans Benefits Administration) as well as local databases. In the national and local databases, 24,014 individuals were identified as having a motor neuron disease (MND) ICD-9 code. Of those individuals, approximately 4,700 medical records were reviewed. An algorithm was developed based on the medical records reviewed to identify ALS cases in the national databases. The algorithm had sensitivity and specificity of 85%. Based on these results, it was determined that it is feasible to create a national ALS registry using national administrative databases. However, the pilot effort also indicated that not all individuals with ALS were in one or more of the national databases. Therefore, ATSDR created an additional secure online portal to gather information about risk factors of ALS not available in the national databases in addition to being able to capture those not in the national databases.