Yes, Lou Gehrig Had Lou Gehrig's Disease

Nov. 19, 2010— -- Catherine Wolf's feet used to point and flex in modern dance class. Now they rest motionless on the footrest of her wheelchair in jeweled ballerina flats.

A matching beaded necklace swoops under a tube that pumps air into her lungs through a hole in her neck. Every 15 minutes, a raspy gargle prompts a nearby nurse to disconnect the tube and suction the build-up of mucous in her lungs. This takes about 10 seconds, during which the smooth, pale skin of Wolf's face turns red like her curly hair and an alarm buzzes loudly.

There's no need for concern, this is routine. It has been 14 years since Wolf, 63, was diagnosed with Lou Gehrig's disease, which has trapped her inside a paralyzed body, silenced her and nearly suffocated her by killing the nerves that control her muscles.

Since Lou Gehrig's famous retirement from baseball in 1939, amyotrophic lateral sclerosis -- the deadly neurological disease that ended the Yankee slugger's 37-year life -- has borne his name. So when an August 2010 research study and the media coverage it garnered challenged the appropriateness of the moniker, it provoked frustration among researchers and confusion and skepticism among patients like Wolf.

"Here he is, the face of his disease, and he may have had a different disease as a result of his athletic experience," study lead author Dr. Ann McKee of Boston University School of Medicine and the New England Veterans Affairs HealthCare System, told a New York Times reporter.

But Dr. Stanley H. Appel, chairman of neurology and director of the Methodist Neurological Institute in Houston, is arguing the claim, calling it a disservice to Gehrig and others with living with ALS.

Appel says there is a lack of scientific evidence to support claims that ''brain trauma can mimic Lou Gehrig's disease'' and that ''maybe Lou Gehrig didn't die of Lou Gehrig's disease," which were printed by The New York Times and Time Magazine respectively. ABC News also covered the study.

"Whether head trauma may have played a role in Gehrig's development of ALS can never be verified, but it is a complete disservice to his place in history as an icon for ALS to suggest that his disease was not ALS," Appel and colleagues wrote in an editorial published in the journal Muscle & Nerve.

What McKee's study actually showed is that chronic traumatic encephalopathy (CTE) -- a condition that stems from repetitive head injuries such as those tolerated by boxers, football players and hockey players -- bears semblance to ALS at the microscopic level. In particular, a protein known to be abnormal in degenerating motor neurons in ALS called TDP-43 also was found to be abnormal in the brains of individuals with CTE -- three of whom went on to be diagnosed with ALS later in life.