Study Calls Lou Gehrig's Disease Into Question

By the end of his impressive baseball career, Lou Gehrig was the face of A.L.S. — or amyotrophic lateral sclerosis — a disease that attacks the brain and the spinal cord. Alan Schwarz of The New York Times and Boston University's Dr. Robert Stern explain the new theories on Lou Gehrig's diagnosis and the disease that bears his name.

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NEAL CONAN, host:

At the end of his career, baseball great Lou Gehrig became indelibly associated with the disease ALS, amyotrophic lateral sclerosis.

(Soundbite of archived speech)

Mr. LHOU GEHRIG: For the past two weeks, you've been reading about a bad break. Today, I consider myself the luckiest man on the face of the earth.

(Soundbite of applause)

Mr. GEHRIG: That I might have been given a bad break, but I've got an awful a lot to live for. Thank you.

CONAN: And everyone since has known it as Lou Gehrig's disease. ALS attacks nerve cells in the brain, and the spinal cord. Victims steadily lose the ability to control their muscles and eventually die. Last week in New York Times, Alan Schwarz wrote about new research that suggests Lou Gehrig might not have had Lou Gehrig's disease, but another condition that stems from brain trauma, from concussions. If you read the story and have questions, give us a call, 800-989-8255. Email us: talk@npr.org. You can also join the conversation on our website. That's at npr.org, click on TALK OF THE NATION.

New York Times' correspondent Alan Schwarz joins us from our bureau in New York City. And, Alan, always nice to have you on the program.

Mr. ALAN SCHWARZ (Correspondent, The New York Times): Well, thank you, Neal. Good to be here.

CONAN: You've been doing a lot of reporting for the Times on brain injuries and athletes. What led you to suspect that this group includes Lou Gehrig?

Mr. SCHWARZ: Well, to be honest, the discovery was made by the same group that have been making a lot of the same - of similar discoveries, brain damage in retired National Foot League players. Excuse me. It's the group out of Boston University and the VA up in New England, which had found that several men who had been diagnosed with ALS, they claim had something else that caused the motor neuron degeneration that looked like ALS to the naked eye, but they say it was something else. And because these men also had such significant histories of brain trauma and the evidence of that trauma - two proteins that are associated with that inside the brain tissue - they believe that this condition was caused by brain trauma, not by, sort of, the inexplicable hand of fate...

CONAN: Mm-hmm.

Mr. SCHWARZ: ...that is often used to explain why ALS happens.

CONAN: Almost a disease whose cause is unknown and whose outcome is certain. And, again, typified by Lou Gehrig, the great strong Iron Horse struck down in the prime of his life.

Mr. SCHWARZ: Well what they claim is - has happened, is that they believe that a small subset, by no means, all or even - excuse me - a majority, but a small subset of people who have been diagnosed with ALS like these three athletes they examined, might have been misdiagnosed. And those with very significant histories of brain trauma, perhaps, those who served in the military, inexperienced combat injuries or, for example, professional athletes in collision sports, if they developed ALS or were diagnosed with ALS, it's possible that they were misdiagnosed, that they have something else.

Now, some people believe that that's a stretch. I had a very nice conversation, yesterday, with a doctor from the Alzheimer's - excuse me - the ALS Association, who disagrees with the finding.

CONAN: Mm-hmm.

Mr. SCHWARZ: But that is what they were saying. Because Lou Gehrig had such a history of brain trauma, they're saying he might have been misdiagnosed too.

CONAN: Brain trauma - getting beaned?

Mr. SCHWARZ: Well, yeah. He was knocked unconscious in a 1934 game and in large part, because he had that long consecutive game streak that we all heard so much about in 1995 when Cal Ripkin broke it, he didn't want to miss the next game. So despite having been knocked unconscious for five minutes, which is a really big deal...

CONAN: Mm-hmm.

Mr. SCHWARZ: ...with a huge lump on his head and a bad headache, he played the next day, because that's what he was supposed to do. That's what he was known for. We know that, now, to be incredibly dangerous. And this happened several documented times in the 1920s and 1930s, when things like that weren't even reported on very much. So it's fair to assume that it happened other times as well. We now know that to be very dangerous.

CONAN: And...

Mr. SCHWARZ: We don't know whether he had ALS, whether he had something else. We don't know really.

CONAN: Well, if this other condition does exists, you would think that in a group of professional athletes whose heads are subjected to injuries a lot -boxers, professional football players - there would be a disproportional number of ALS diagnosis.

Mr. SCHWARZ: In fact, there is. There have been 14 or 15 retired NFL players diagnosed with ALS, which is roughly eight times what would be expected in that population of that age group. And there really only should have been one or two, when there have been 14 or 15. Now, that means two things. One, that this is real, that there is something going on. And two what it also means is it's not affecting that many people. I mean, 12 or 13 extra guys in 50 years - you know, it matters a lot to those 12 or 13 guys and their families, don't get me wrong.

CONAN: Mm-hmm.

Mr. SCHWARZ: But by no means should this finding be confused with the far more widespread problem of post-concussion syndrome, second-impact syndrome, and some of the dementia and cognitive decline problems that have been associated with head impacts on the football field. That affects thousands of people.

CONAN: Also with us today is Dr. Robert Stern, associate professor of neurology and codirector of Alzheimer's Disease Clinical and Research Program at Boston University, cited Alan Schwarz' piece last week in The New York Times. Dr. Stern, thanks very much for being with us today.

Dr. ROBERT STERN (Codirector of Alzheimer's Disease Clinical and Research Program, Boston University): Great to be here, Neal.

CONAN: And you're quoted in the piece saying, "People are being misdosed(ph) clinically while they're alive as having ALS when they, in fact, have a different motor neuron disease. ALS is famously difficult to treat. Is this other motor neuron disease treatable?

Dr. STERN: No. Unfortunately, it's not treatable. And also the concept of misdiagnosis needs to be clarified. The diagnosis of ALS is a clinical diagnosis that is made by many different tests that neurologists would be conducting on a patient. And there are many different types of ALS. They might all be considered motor neuron diseases. And the most common type of motor neuron disease is indeed ALS. But most of those motor neuron diseases are going to be considered ALS, clinically. And it's only when you look at the brain and the spinal cord under a microscope, that it can truly differentiate amongst these diseases - including this new disease that we have described in this recent paper.

CONAN: When you look at them under a microscope, is that possible early on in the diagnosis or is that only in an autopsy?

Dr. STERN: Unfortunately, it is only after the person passes away and we look at their brains after an autopsy.

CONAN: And do the diseases present - or the conditions - present differently, or they're diagnosed as ALS because they look very much like ALS?

Dr. STERN: They are all potentially considered ALS, even. Many clinicians who are the world's greatest ALS experts will diagnose all of these various types of diseases, these motor neuron diseases, as ALS. And this is really talking about one of the potential causes of these diseases. So it looks different under a microscope. And more than likely, it has a different cause or a specific cause, different from sporadic ALS and the other types of ALS. So in this case, the cause is likely to be repetitive head trauma that these athletes had survived during their life. But there are many other causes of ALS, from genetics to, perhaps, neurotoxins, to who knows what. Most of the causes are not yet known, and there are scientists trying to figure it out. What this new disease is saying is that there's likely one cause that we are now understanding, and that's repetitive head trauma.

CONAN: We have a caller on the line. Cynthia(ph) is with us from Spokane in Washington.

CYNTHIA (Caller): Hi. Thank you for taking my call.

CONAN: Go ahead, please.

CYNTHIA: I'm a widow, someone who is diagnosed with ALS. And I am aware of the different variations of what people consider ALS, like one of speakers were saying. It was at the time my husband passed away, which is basically about five years ago. We were living in Palm Springs. And like your speaker talked of, the percentage of people that we run into who knew someone who had ALS was amazingly higher than what we hear and read on the websites for information. But my question is really, regarding brain trauma mimicking. I would like to know exactly how far brain trauma - this kind of - the symptoms can mimic ALS. Would it be until death? Is - do we have - is there a lateral disintegration of muscles? And like ALS, does the patient fail to be able to swallow and breathe and things like that in that order?

CONAN: Dr. Stern, can you help us out?

Dr. STERN: Yeah. I think it's fair to say that - it not only mimics ALS, it would be appropriately diagnosed as ALS. This new disease is another form of ALS or motor neuron disease that will look the same, will have the same clinical presentation, the same course as this woman's husband unfortunately suffered through. And it's really - it's a matter of scientific knowledge, how we look at the brain tissue after someone passes, to understand the different types and forms and causes of ALS. And so, clinically, they're not going to be distinguishable.

CONAN: Cynthia, we're very sorry for your loss, and thank you very much for the phone call.

CYNTHIA: Thanks for your time, and thanks for this subject.

CONAN: We're talking with, as you just heard, Dr. Robert Stern at Boston University. And also with us, Alan Schwarz, a correspondent for The New York Times. You can find a link to his piece, "Study Says Brain Trauma Can Mimic ALS," there's a link to it on our website at npr.org, click on TALK OF THE NATION. And you're listening to TALK OF THE NATION from NPR News.

And we have this email question from Debbie(ph) in Columbus: I nearly died from a brain injury 25 years ago. How long does it take before ALS-like symptoms would appear? And Dr. Stern, just going back to something that Alan said earlier, Debbie, it would seem to me, might - would Debbie be more at risk from the ALS-like symptoms or more at risk from the cognitive problems that Alan was talking about earlier?

Dr. STERN: Yeah, it's a good question. The big issue is that when you hit your head over and over again, as seen in the military and in collision sports, it obviously isn't going to be good for you. But not everyone who has a brain trauma, either a single one, perhaps like Debbie's, or the repetitive nature of collision sports, not everyone who has these repetitive brain traumas will go on to develop these diseases later in life. We don't yet know who is at risk for developing these diseases.

Like Alan was saying earlier, we do know that there's an increased risk of developing what we call chronic traumatic encephalopathy, or CTE, which is a cause of dementia later in life that is due to repetitive head trauma earlier in life. But that doesn't mean that everyone who hits their head will go on to develop CTE. Even fewer people will go on to develop this ALS-like disease later in life.

So just because someone has had a brain injury or they've had even repetitive head trauma, that doesn't mean they should be scared that they're going to develop these diseases. They're relatively rare, and we don't yet know what else adds to the kind of magic combination of risks that put people at a greater chance of getting the disease later.

CONAN: Alan Schwarz, let me get back to you just for a moment. It's interesting, you've been doing a groundbreaking series of reports on injuries, most of them about the National Football League, professional football.

Earlier this week the owners of the National Football League met to consider the idea of expanding from 16 to 18 regular season games per year. And I wonder, can you see that there's been any effect of the news of the effect of repetitive head injuries on how the league is designing equipment or designing its game?

Mr. SCHWARZ: Well, I think that you have to - one would have to consider the proposal of extending the season from 16 to 18 games solely in the context of collective bargaining. The owners want to have two more games in order to make, you know, 12 percent more money. And the players don't want that, or if they say that's okay, then they want a very high cut of the extra money. It's all collective bargaining.

Now, the league, after spending three years or so doing considerably less about head injuries and concussions and sub-concussive blows, did do several things at the end of last year, requiring an independent neurologist to decide whether a player can go back to a practice or to a game and then ultimately not even allowing players who sustain a concussion or believe to have had a concussion to go back into the same game or practice. These are things that the league defended publicly only months before.

They have done some very, you know, good things regarding their place in terms of leaders - thought leaders in the industry. I just(ph) think that if they were to take two pre-season games and make them regular season, that's a collective bargaining issue. It has very little to do with player health.

CONAN: Well, let's see if we can get a call in. Dennis(ph) is on the line from Bryant in Arkansas.

DENNIS (Caller): Yes. I can't remember the name of the English astrophysicist whose worldwide known case...

CONAN: I think it's Stephen Hawking.

DENNIS: Yeah, Stephen Hawking. Of course, I knew. He survives such a long time, I was wondering if there's anything else atypical about his - how was he diagnosed as ALS? Is it absolutely certain that's what he has?

CONAN: Dr. Stern?

Dr. STERN: Yeah, it - I can't imagine that he doesn't have ALS, and he is definitely an outlier. Most people with ALS will sadly pass away within a few years. And very few people do have the long life that Stephen Hawking has shown.

It's also important to note that the majority of people with sporadic ALS get it later in life, perhaps in their 50's and 60's or even later. And what it appears like is that these athletes, as well as people in the military, seem to get this other disease, ALS-like disease perhaps, or ALS itself earlier in life, perhaps in their 40's.

CONAN: Dennis, thanks very much for the phone call.

DENNIS: Yeah. Thanks.

CONAN: Appreciate it. And Dr. Stern, thank you very much for your time today.

Dr. STERN: It's my pleasure.

CONAN: Robert Stern is director of Clinical Core, a research registry for patients with Alzheimer's disease. He joined us from the studios at Boston University. Alan Schwarz, correspondent for The New York Times. You can read his article - again, there's a link to it at npr.org, click on TALK OF THE NATION - joined us from our bureau in New York. Alan, as always, thanks very much.

Mr. SCHWARZ: My pleasure, Neal.

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