Posted 6/30/2004 10:04 PM
© Copyright 2004 USA TODAY, a division of Gannett Co. Inc.
By Kathleen Fackelmann, USA TODAY

Lou Gehrig was a star first baseman for the New York Yankees in 1939 when he felt the first symptoms of amyotrophic lateral sclerosis, which would later take on his name. Two years later, at 37, Gehrig was dead.

More than 60 years later, scientists still have no cure for ALS, or Lou Gehrig's disease, even as the scientific progress on the fatal neurological disease has virtually exploded.

But that basic research has yet to lead to a drug that significantly slows the disease, which often kills within three to five years, says Scott Heller, a neurologist at the Northwestern Memorial Hospital in Chicago.

"This is still a pretty heinous disease," he says. Up to 30,000 Americans have ALS, a disease that kills brain cells called motor neurons.

Scientists say most cases are caused by a complex interplay of unknown genetic and environmental factors. Researchers have identified a gene that is linked to ALS for a handful of cases in which the disease runs in families. Aside from those, there is no test for Lou Gehrig's disease. To identify the disease, doctors must rule out a lot of other conditions.

At first, the disease causes twitches and cramping, symptoms that are easy to ignore, says Valerie Cwik, medical director of the Muscular Dystrophy Association in Tucson. As the disease progresses, patients lose the ability to move their arms, legs or other parts of the body.

In the end, family members have to help bedridden patients perform basic activities of daily life. Most patients die of suffocation when the disease strips the chest muscles of the ability to move air in and out of the lungs.

"This disease takes a huge toll on patients and their families, because they know what's coming down the road," Cwik says.

But progress on the scientific front might soon change that outlook. Doctors already have riluzole, a drug that prolongs survival of people with ALS by about three months. That drug works by clearing excess glutamate, a chemical messenger thought to play a role in ALS, says Lucie Bruijn of the ALS Association in Calabasas Hills, Calif.

No one drug is likely to offer ALS patients a cure anytime soon. Yet Bruijn and other experts say research has suggested a number of potential therapies. For example, researchers are testing several drugs thought to work by curbing the inflammation that occurs with the disease.

Such research will lead to one or more drugs that might buy patients more time, Bruijn says.

"We will have drugs within the next 10 years that will dramatically slow the disease," she says.