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Novel Protein Test May Predict Progression Rate Of Amyotrophic Lateral Sclerosis

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Novel Protein Test May Predict Progression Rate Of Amyotrophic Lateral Sclerosis

According to a study, which appears online in the Journal of Neurology, Neurosurgery & Psychiatry, researchers from Mayo Clinic’s campus in Florida, Emory University and the University of Florida, were able to create a novel test which may reveal the rate of progression of amyotrophic lateral sclerosis (ALS). This test measures the levels of proteins from nerve damage that are deposited in blood and spinal fluid and could represent a useful method for both physician and researches in identifying patients with amyotrophic lateral sclerosis that have a higher risk for rapid progression.

Amyotrophic lateral sclerosis, also known as Lou Gehrig's disease, is a progressive neurodegenerative disorder caused by a progressive loss (reduction) of certain nerve cells, of the  brain and spinal cord, called motor neurons. Motor neurons control voluntary muscles, muscles that make movement possible. Amyotrophic lateral sclerosis is a progressive, debilitating, and a fatal disease. Walking, talking, eating, swallowing and other basic functions become more difficult with time. These conditions can cause different injuries, illness and other problems. Disease progression rate varies wildly among patients and for this reason, survival from the date of diagnosis can be months to more than 10 years.

“In the care of our ALS patients there is a need for more reliable ways to determine how fast the disease is progressing. 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.”, says Dr. Boylan, study’s lead investigator.

Blood test

Blood test

Until today, there are no curative therapies for ALS, but many studies related to this issue are in progress. Actual treatment for Lou Gehrig's disease can help maintain stability and independence, keep symptoms under control and avoid complications as much as possible. Scientists believe that a test like this is needed because, on the one hand, may identify patients with risk for a faster progression of muscle weakness and on the other hand, they may detect a faster response to experimental drugs that primarily slow progression of ALS in patients with rapid progressive forms of the disease. Study’s lead investigator also believe that if exists a way to identify patients who are at risk for having a faster progression of amyotrophic lateral sclerosis, it should be possible to conduct therapeutic trials with a smaller number of patients in a shorter period of time than is required presently. Another goal for researchers is to develop tests like this in order to gauge how well a patient is responding to experimental therapies.

This novel test measures neurofilament heavy form from blood and spinal fluid. Neurofilament heavy form are proteins that confer structure to motor neurons, and in the moment when motor neurons are denatured by the disease, this proteins break down and accumulate in spinal fluid and blood serum. In this study, scientists measured nurofilament heavy form in blood and spinal fluid and observed that in ALS patients with rapid progression of muscle weakness the levels of this proteins were higher than in patients with a slow evolution. They also observed that patients with amyotrophic lateral sclerosis and a higher level of neurofilament heavy form have a shorter survival.