ALS (amyotrophic lateral sclerosis), a motor neuron disease, was first identified in 1869 by the noted French neurologist Jean-Martin Charcot. Lou Gehrig, with whom ALS is most commonly associated, first brought national and international attention to the disease back in 1939 when he abruptly retired from baseball after being diagnosed with ALS.
However, ALS is not just Lou Gehrig’s disease and it knows no boundaries. The disease has cut short the lives of such notable and courageous individuals as Hall of Fame pitcher, Jim “Catfish” Hunter; actor, Michael Zaslow; creator of Sesame Street, Jon Stone; actor, David Niven; boxing champion, Ezzard Charles; pro football player, Glenn Montgomery; and Senator Jacob Javits.
A little over 5,000 people in the U.S. are diagnosed with ALS each year, or 14 new cases a day. It is estimated that as many as 30,000 Americans have the disease at any given time. Most people who develop ALS are between the ages of 40 and 70, with an average age of 55 at the time of diagnosis. ALS occurs throughout the world with no racial, ethnic, or socioeconomic boundaries, and affects males and females equally. Half of all people affected with ALS live at least three or more years after diagnosis. Twenty percent live five years or more; up to ten percent will survive more than ten years.
ALS is a progressive neurodegenerative disease that attacks nerve cells in the brain and the spinal cord. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With all voluntary muscle action affected, patients in the later stages of the disease become totally paralyzed. Yet, through it all, for the vast majority of people, their minds remain unaffected. Rilutek®, the first treatment to alter the course of ALS, was approved by the FDA in late 1995. This antiglutamate drug appears to prolong the life of persons with ALS by at least a few months and more recent studies suggest Rilutek slows the progress of ALS, allowing the patient more time in the higher functioning states. Rilutek® is manufactured by sanofi-aventis.