TDP-43 Proteinopathy and Motor Neuron Disease

Researchers from Boston University are publishing a new study in the Journal of Neuropathology and Experimental Neurology on the relationship of head trauma and amyotrophic lateral sclerosis (ALS).  The researchers found toxic proteins in the spinal cords of the three athletes.  Those same proteins have been found in the brains of athletes with chronic traumatic encephalopathy (CTE), a disease linked to head injuries that causes cognitive decline, abnormal behavior and dementia.

Previous studies have linked TAR DNA-binding protein (TDP-43) to some familial forms of the disease, and although etiology of sporadic ALS is unknown, it has long been suspected to involve a complex interaction between multiple genetic and environmental risk factors.  Among the many environmental factors that have been considered as possible triggers of the neurodegenerative cascade in ALS are a history of trauma to the brain and spinal cord, participation in varsity athletics and a slim physique, and strenuous physical activity.

A New York Times article suggests that brain trauma can mimic ALS, and that perhaps Lou Gehrig may not have had the disease that bears his name.  ALS Association Chief Scientist Lucie Bruijn is quoted in the article as saying, “It’s extremely interesting - it builds a more interesting picture, but what this all exactly means about how the disease plays out requires further investigation.”

This new study adds to the body of knowledge surrounding motor neuron disease, and points out that more research using a greater number of cases are required to produce conclusive results.

To read the full New York Times article, visit http://www.nytimes.com/2010/08/18/sports/18gehrig.html?_r=1&emc=na