ALS, or Lou Gehrig's disease, is a rare, difficult to diagnose neurological condition with no known cause or cure. Because ALS is a non-notifiable disease, little is known about its incidence and prevalence in the U.S. To help learn more about ALS, Tracking California collaborated with the Agency for Toxic Substances and Disease Registry (ATSDR) to conduct retrospective surveillance in Los Angeles County (LA) and five counties in the San Francisco Bay Area (SFBA).
The project found that general case demographics and incidence rates of ALS cases in these two areas were similar to published studies. However, the differences between the two areas raise questions about how factors such as geography, access to care, and referral patterns affect case ascertainment and diagnosis. This project informs the larger national ALS surveillance effort, which is an important next step for better understanding the current epidemiology of ALS in the U.S. population.
ALS is a fatal, rapidly progressive neurological disease. Few population-based surveillance systems and registries exist for neurological diseases such as ALS, making calculation of incidence rates and impact assessment difficult. In 2008, Congress mandated that the Agency for Toxic Substances and Disease Registry (ATSDR) develop a registry to gather and organize information about people living with ALS. ATSDR funded multiple state and metropolitan area-based surveillance projects to validate the completeness and accuracy of data in the National ALS Registry. As part of this nationwide surveillance effort, retrospective surveillance was conducted in two diverse metropolitan areas: Los Angeles County (LA) and the San Francisco Bay Area (SFBA).
The objectives of this effort were:
Primary Data Collection
Neurologists were contacted to determine if they diagnosed or cared for ALS patients and were asked to submit one-page case reporting forms for SFBA or LA residents with ALS under their care from January 1, 2009 through December 31, 2011. Case reports included demographic and clinical data. A medical record verification form and a copy of an electromyogram (EMG) report were requested for a sample of cases.
Secondary Data Collection
Using California's Electronic Death Registration System (EDRS), staff obtained records of all deaths among residents of Los Angeles County and the five Bay Area counties from January 1, 2009 through December 31, 2011 and identified decedents with ALS as one of the causes of death. These deaths were matched to cases reported through primary data collection, and those deaths from ALS that remained unmatched were followed up; Physicians of record in the death record were contacted to verify diagnosis and obtain an ALS case report.
ALS surveillance in two ethnically diverse California metropolitan areas successfully identified at least 94% of expected cases.
View final project report and project summary results for more details.
Project summaries and results for all participating states and metro areas.
Congressionally mandated registry for persons in the United States with ALS. It is the only population-based registry in the country that collects information to help scientists learn more about who gets ALS and its causes.
National non-profit organization conducting research, providing assistance for people with ALS through a nationwide network of chapters, coordinating multidisciplinary care through certified clinical care centers, and fostering government partnerships.
Local chapter of the national ALS assocation covering the Greater Los Angeles and Greater Bay Area. ALSA Golden West conducts research, provides assistance for people with ALS throughout the state, coordinates multidisciplinary care through certified clinical care centers, and fosters government partnerships.
Non-profit health agency dedicated to finding treatments and cures for ALS, muscular dystrophy, and other neuromuscular diseases. This association funds research, provides comprehensive health care services and mobilizes communities for advocacy.
An international, independent, non-profit group of researchers who collaboratively conduct clinical research in ALS and other motor neuron diseases. NEALS mission is to translate scientific advances into new treatments for people with ALS and motor neuron diseases as rapidly as possible.