Protein Test is First to Predict Rate of Progression in Lou Gehrig's Disease

A novel test that measures proteins from nerve damage that are deposited in blood and spinal fluid reveals the rate of progression of amyotrophic lateral sclerosis (ALS) in patients, according to researchers from Mayo Clinic's campus in Florida, Emory University and the University of Florida.

Their study, which appears online in the Journal of Neurology, Neurosurgery & Psychiatry, suggests this test, if perfected, could help physicians and researchers identify those patients at most risk for rapid progression. These patients could then be offered new therapies now being developed or tested.

ALS - also known as Lou Gehrig's disease - is a progressive neurodegenerative disease caused by deterioration of motor neurons (nerve cells) that control voluntary muscle movement. The rate of progression varies widely among patients, and survival from the date of diagnosis can be months to 10 years or more, says Kevin Boylan, M.D., medical director of the ALS Clinic at Mayo Clinic in Florida.

"In the care of our ALS patients there is a need for more reliable ways to determine how fast the disease is progressing," says Dr. Boylan, who is the study's lead investigator. "Many ALS researchers have been trying to develop a molecular biomarker test for nerve damage like this, and we are encouraged that this test shows such promise. Because blood samples are more readily collected than spinal fluid, we are especially interested in further evaluating this test in peripheral blood in comparison to spinal fluid."

There are no curative or even significantly beneficial therapies in clinics now for ALS treatment, but many are in development, Dr. Boylan says. A test like this could help identify those patients who are at risk for faster progression of weakness. With experimental treatments that primarily slow progression of ALS, detecting a treatment response in patients with faster progression may be easier to detect, says Dr. Boylan. Now, patients with varying rates of progression participate together in clinical studies, which can make analysis of a drug's benefit difficult, he says.

Lou Gehrig's disease, also called amyotrophic lateral sclerosis (ALS), causes the nerve cells in certain regions of your brain and spinal cord to gradually die. Eventually, people who have Lou Gehrig's disease (ALS) lose the ability to move their limbs and the muscles needed to move, eat, speak and breathe.

Doctors don't know what causes Lou Gehrig's disease, and the disease can't be cured. Although doctors can't reverse the progression of Lou Gehrig's disease, advances in treatment mean that many who have the disease live longer than they did in the past. Each year about 5,000 Americans are newly diagnosed with ALS, and about 20,000 Americans have the disease.

Lou Gehrig's disease first gained widespread public attention in the United States in 1939. Doctors at Mayo Clinic diagnosed baseball player Lou Gehrig's condition.