Proposal for State and Metropolitan Area-Based ALS Surveillance

Amyotrophic lateral sclerosis (ALS or Lou Gehrig disease) is a neurodegenerative disorder that affects people of all ages, most commonly those in their 50s through 70s. The prognosis is poor, with more than half of patients dead from respiratory muscle paralysis within 3 years of diagnosis, and only a small portion surviving more than 5 years. It can be a genetic disease, although most occurs sporadically. ALS can be difficult to diagnose accurately due to its insidious onset, varied presentation, and the lack of a definitive marker for it. The relationship of location and related environmental factors has never been established. In 2008 Public Law No 110-373 established a national ALS Registry. The Centers for Disease Control (CDC) have designed a case identification strategy using data from Medicare, Medicaid and the Veterans Administration. In order to evaluate the completeness of ascertainment and accuracy using that approach, they have also developed a 2-3 state-based and 4-6 metropolitan area-based surveillance protocol for ALS. Our program has been approached to be the surveillance site for Cook and DuPage counties, since we see approximately 80% of all ALS cases in the greater Chicago metropolitan area. We are being asked to provide basic information, including some that can identify a patient, as well as a brief summary of supporting evidence for the diagnosis, for each ALS case being seen in the clinics in the Division of Neuromuscular Medicine from Jan1, 2009 through Dec 31, 2011. This data will be reported to the Metropolitan Chicago Healthcare Council-Chicago Hospital Council, who will in turn forward the information to CDC. The duration of the project is one year.