A growing collection of anecdotal stories raises the possibility that nerve injury in an arm or a leg can act as a trigger for the development amyotrophic lateral sclerosis, or ALS--a progressive neurodegenerative disease also known as Lou Gehrig's disease, named after the famous New York Yankee who died of it in 1941. The connection between ALS and athletes runs deeper than a single ballplayer; people who engage in intense physical activities, such as professional athletes and people in the military, are more likely to be affected by ALS. In some, the disease seems to start after an injury -- muscle weakness at the site of the injury slowly spreads to new areas until weakness in the muscles responsible for breathing causes suffocation. Now, researchers at the University of Illinois at Chicago (UIC) are the first to demonstrate that a peripheral nerve injury can trigger the onset and spread of the disease in an animal model of ALS. Their findings, published in the April 2019 issue of Neurobiology of Disease, show that rats genetically engineered to develop ALS-like symptoms have an abnormal inflammatory response in the region of the spinal cord associated with an injured peripheral neuron. As the spinal cord inflammation and other damaging processes spread, they cause progressive muscle weakness throughout the body. The article is titled “Mutant SOD1 Prevents Normal Functional Recovery Through Enhanced Glial Activation and Loss of Motor Neuron Innervation After Peripheral Nerve Injury.” "We know that in some patients with ALS the weakness starts in a hand or leg, and the disease spreads. Coincidentally, the patient will describe a recent or remote injury to that same hand or leg that matches the location of their disease onset.
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