Protein test is first to predict rate of progression in Lou Gehrig's disease

November 19, 2012

JACKSONVILLE, Fla. -- 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.

"If there were a way to identify people who are likely to have relatively faster progression, it should be possible to conduct therapeutic trials with smaller numbers of patients in less time than is required presently," Dr. Boylan says.

A longer-range goal is to develop tests of this kind to gauge how well a patient is responding to experimental therapies, he adds.

The test measures neurofilament heavy form in blood and spinal fluid. These are proteins that provide structure to motor neurons, and when these nerves are damaged by the disease, the proteins break down and float free in blood serum and in the spinal fluid. Earlier research in this area was conducted by Gerry Shaw, Ph.D., a neuroscientist at the University of Florida, who is the study's senior investigator and the developer of the neurofilament assay used in the study.

The researchers measured neurofilament heavy form in blood and spinal fluid samples from patients at Mayo Clinic and at Emory University, and correlated levels of the protein with rate of progression. "We demonstrated a solid association between higher levels of this protein and a faster progression of muscle weakness," Dr. Boylan says. There was also evidence suggesting that ALS patients with higher protein levels may have shorter survival, he adds.
-end-
The work was funded by the ALS Association, the Packard Center for ALS Research, MCF ALS Center Donor Funds and Mayo Foundation.

About Mayo Clinic

Mayo Clinic is a nonprofit worldwide leader in medical care, research and education for people from all walks of life. For more information, visit www.mayoclinic.org/about/ and www.mayoclinic.org/news.

Mayo Clinic

Related Amyotrophic Lateral Sclerosis Articles from Brightsurf:

Converting lateral scanning into axial focusing to speed up 3D microscopy
In optical microscopy, high-speed volumetric imaging is limited by either the slow axial scanning rate or aberrations introduced by the z-scanning mechanism.

Ammonium triggers formation of lateral roots
Despite the importance of changes in root architecture to exploit local nutrient patches, mechanisms integrating external nutrient signals into the root developmental program remain poorly understood.

'Reelin' in a new treatment for multiple sclerosis
In an animal model of multiple sclerosis (MS), decreasing the amount of a protein made in the liver significantly protected against development of the disease's characteristic symptoms and promoted recovery in symptomatic animals, UTSW scientists report.

Adjustable lordotic expandable vs static lateral lumbar interbody fusion devices
The objective of this study is to compare the clinical and radiographic outcomes between patients treated with static and expandable interbody spacers with adjustable lordosis for MIS LLIF.

Chirality-assisted lateral momentum transfer for bidirectional enantioselective separation
Chiral nanoparticles which twist the light were theoretically predicted to experience lateral forces perpendicular to light vector but lacks experimental verification.

Not all multiple sclerosis-like diseases are alike
Scientists say some myelin-damaging disorders have a distinctive pathology that groups them into a unique disease entity.

Researchers delay onset of amyotrophic lateral sclerosis (ALS) in laboratory models
Scientists have delayed the onset of amyotrophic lateral sclerosis (ALS) in laboratory models, leaving them cautiously optimistic that the result, combined with other clinical advances, points to a potential treatment for ALS in humans.

Emerging role of adenosine in brain disorders and amyotrophic lateral sclerosis
The role of adenosine in neurodegeneration and neuroregeneration has led to growing attention on adenosine receptors as potential drug targets in a range of brain disorders, including neuroregenerative therapy and treatment for amyotrophyic lateral sclerosis (ALS).

New clues about the origins of familial forms of Amyotrophic lateral sclerosis
A Brazilian study made important progress in understanding the accumulation of one of the proteins involved in amyotrophic lateral sclerosis (ALS).

Recrutement of a lateral root developmental pathway into root nodule formation of legumes
Peas and other legumes develop spherical or cylindrical structures -- called nodules -- in their roots to establish a mutually beneficial relationship with bacteria that convert atmospheric nitrogen into a useable nutrient for the legume plant.

Read More: Amyotrophic Lateral Sclerosis News and Amyotrophic Lateral Sclerosis Current Events
Brightsurf.com is a participant in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising fees by advertising and linking to Amazon.com.