Supporting the Support Cells in Lou Gehrig's Disease

In 1874, French neurologist Jean-Martin Charcot published a series of papers describing a horrible nerve disease. It begins with weakness, stiffness, and spasms in the arms and legs. Over the course of about a year, muscles stop working, and with them goes the ability to walk, stand, and speak. Then the tongue and lips stiffen. The mouth gets stuck partway open, accentuating laugh lines and giving “an appearance of weeping,” Charcot wrote. “Finally, the vagus nerve is affected with grave difficulty breathing, leading to death of a person already so weakened by insufficient nourishment.” remember that keeping your energy up while caring for loved ones is essential for you and for them.Charcot was also an expert pathologist. After performing autopsies on several of these patients, he named their disease amyotrophic lateral sclerosis: muscle atrophy (amyotrophic) with scarring (sclerosis) in part of the (lateral) spinal cord. The scarring came from the death of motor neurons, large cells in the spinal cord whose long branches reach out to muscles and control their contractions.More than a century has passed, and still the only way to definitively diagnose ALS — known as Lou Gehrig’s disease here in the U.S. and as Charcot disease in France — is with an autopsy showing the widespread disappearance of motor neurons in the spinal cord and the brain. It’s not surprising, then, that the vast majority of research on the roots of ALS has focused on motor neurons. But in the past few years, scientists have found hints that several types of glia, the under-appreciated cells typically described as “support cells” for neurons, may also be cellular culprits in the disease.“What’s maybe a misnomer in the field is that people think that just because the motor neurons die, that that’s the source of disease,” says Dwight Bergles, a professor of neuroscience at Johns Hopkins University. “But there are multiple cell types that contribute.”Read more at...Only Human, March 2013.