Lou Gehrig's disease an extremely rare malady, CDC says

Lou Gehrig, the iconic New York Yankee slugger, was forced to retire from baseball in 1939 because of a rare neuromuscular disorder. New data from the Centers for Disease Control and Prevention gives a first-of-its-kind glimpse into just how rare that incurable disease is.

The CDC's Agency for Toxic Substances and Disease Registry found that a little more than 12,000 people (PDF) between October 2010 and December 2011 were living in the U.S. with amyotrophic lateral sclerosis—better known as Lou Gehrig's disease. That amounts to about four in 100,000 people. The CDC said ALS, which destroys nerve cells and leads to paralysis, usually leads to death within two to five years of diagnosis.

ALS most commonly affects older white males, according to the data. White men and women were also twice as likely to have ALS as were black men and women.

The CDC and other advocacy groups are hoping the results will prompt more comprehensive data collection in the future, since ALS does not have to be reported in each state. Massachusetts is the only state that requires reporting of ALS.

“This initial report is an important milestone for the National ALS Registry and adds to our knowledge of the disease,” said Barbara Newhouse, president of the ALS Association. “But this is just the beginning. The first step in what is a long-term research project that not only can tell us how many people have ALS, but more importantly, why they have it. Further enrollment of people living with ALS is critical.”

The National ALS Registry was established in 2008 (PDF) to aggregate data on people with the disease. The registry relies on self-reporting and data from Medicare, Medicaid and the Veterans Affairs Department.

Follow Bob Herman on Twitter: @MHbherman