Peter Frampton on Rockin’ for Research to Cure Inclusion Body Myositis

Episode Transcript

Dr. Daniel Correa:
Saludos and hello, I'm Daniel Correa.

Dr. Audrey Nath:
And I'm Audrey Nath.

Dr. Daniel Correa:
We're two neurologists and fellow brain geeks hosting the Brain & Life Podcast. This show, the Brain and Life Magazine and website are all brought to you by the American Academy of Neurology.

Dr. Audrey Nath:
We're so glad you're joining us. We'll be here each week for conversations and interviews with community members, celebrities, and experts on brain health and living with neurologic conditions. Now let's get to today's show. Daniel, you're going to be jealous. I got to speak with an actual rock star legend, Peter Frampton.

Dr. Daniel Correa:
Peter Frampton? Oh, wow.

Dr. Audrey Nath:
Yeah. Growing up, I knew him from The Simpsons, but you may know some of his songs like Baby, I Love Your Way.

Dr. Daniel Correa:
"Ooh, baby I love your way."

Dr. Audrey Nath:
That is amazing. Oh my goodness. You should join him on tour. He's on tour.

Dr. Daniel Correa:
Yeah, I grew up with the car rides, my dad and my uncle, they both pretty much lived with classic rock stations on almost all the time. That was sort of the soundtrack of me going back and forth to soccer practices and wrestling in school as I was growing up.

Dr. Audrey Nath:
It's incredible, Peter Frampton's been around for decades with such a storied career and albums, and he's a guitar genius. And it was just fascinating to speak with him about what it's been like to have a muscle disorder. So inclusion body myositis, which caused some weakness starting in adulthood. And just so interesting to talk to him about how he's continued with songwriting and how he's adapted with continued to play guitar and is even going on tour one last time now.

Dr. Daniel Correa:
Wow, I'm really looking forward to hearing how he just gets on tour and how he moves through with, despite this diagnosis.

Dr. Audrey Nath:
Today, we are in the presence of a legend. Peter Frampton is the rock star with a critically acclaimed multi-decade career as a guitarist, singer, and songwriter. His album, Peter Frampton Comes Alive, is one of the best selling live albums of all time. Many of us know him from numerous hits on Classic Rock Radio and for any 90s kids like me, I first saw Peter Frampton on The Simpsons on the Lollapalooza episode. So I know you as a yellow cartoon character singing and then getting upset that your big floating pig flew away.

Peter Frampton:
That's right.

Dr. Audrey Nath:
That's you. More recently around 10 years ago, Peter started noticing some weakness and was ultimately diagnosed with a muscle condition called inclusion body myositis or IBM. Briefly, myositis means muscle inflammation, and the inclusion body part refers to how the muscle cells look under a microscope with unusual inclusions of protein clumps. Yeah, I know. Talking about what your muscle cells look like under a microscope isn't nearly as exciting as seeing you perform in a stadium, but we want to highlight the incredible work that Peter has done with the Peter Frampton Myositis Research Fund at John Hopkins to help improve the lives of people with this condition and find new treatments. Welcome to the Brain & Life Podcast, Peter Frampton.

Peter Frampton:
Oh, thank you so much. Thank you, Audrey.

Dr. Audrey Nath:
Oh, we want to hear in your own words for anyone that doesn't know this story. When did you start to notice that something was changing with your body? What happened?

Peter Frampton:
Well, the very first thing was quite a few years ago, maybe 14 years ago, I thought about this the other day. Just before I was going to go on stage, one of my fingers just had a convulsion, and I felt it was just retracting and I had to massage it. I'm standing with guitar on, waiting to go on. So I didn't-

Dr. Audrey Nath:
Oh, boy.

Peter Frampton:
Well, I'm sure it's because I used my hands too much. So that's one thing. But the main thing was about 12 years ago I guess, is when I first noticed something, and it was my son and I went up to Big Sur, we stayed at the Big Sur Lodge, we did a dad and son four day weekend up there. It was wonderful.

Dr. Audrey Nath:
That's awesome.

Peter Frampton:
And we wrote songs together, and we hiked together, and we did all that good stuff that you do because he's a player too. But there was this one hill that he ran up, and I tried, I would usually have beaten him, he disagrees there, but I got to the top and I said, "You know what? I'm getting old. This is hard for me." And that was the first symptom that I was noticing that I had.

Peter Frampton:
And then after that I went on tour, and we were playing the band after soundcheck, we're just staying at the gig and throwing a frisbee around the backstage area. And I was finding it very hard to run for the frisbee. And I said, "Something's going on here." So anyway, the way you feel to begin with is, "Ah, I'm 60 now. I'm getting old. I'm 59," whatever it was. And then I believe it was on that tour about 11 years ago now, I guess, I fell on stage. I kicked a beach ball, one of those huge ones that came up on stage, it was an outdoor gig. And of course, I kicked it with my right foot and with IBM, one side is weaker than the other usually.

Dr. Audrey Nath:
Oh, boy.

Peter Frampton:
And so I'm supporting myself on my left leg, which is my weakest one.

Dr. Audrey Nath:
Oh, geez.

Peter Frampton:
And I went backwards. So I fell down. So we all thought that was really funny. Me included, even though it was slightly humiliating. And we got on the bus and then two to three weeks later, unfortunately, I bent down on stage and so my chord from my guitar shortened. I stepped on it, and then I went to stand up, and it pulled me backwards again. So, we had a 10 day break coming up within a few days at that point. And I said, "I need to see a neurologist."

Dr. Audrey Nath:
Yeah. That'll do it.

Peter Frampton:
Yeah. So one of you guys.

Dr. Audrey Nath:
Yeah, exactly. We get that a lot.

Peter Frampton:
So anyway, my GP in Nashville, I told him, and he was worried I'd lost 10 pounds since I'd last seen him, which was a few years actually. He said, "Something's going on here." And he said, "I think, with the weakness, I'm going to have you see this neurologist that I know." Well, made an appointment, and he diagnosed me correctly the first time, which is very unusual for IBM.

Dr. Audrey Nath:
Yes, it is tricky to diagnose because it looks like several other conditions, including ALS.

Peter Frampton:
I know.

Dr. Audrey Nath:
Which is a condition that has a high fatality rate. Were you worried about that?

Peter Frampton:
Well, I didn't know until he told me that.

Dr. Audrey Nath:
Okay.

Peter Frampton:
So he said, "I have good news for you because my first thought was it was ALS. If that was the case, I'd be telling you to get your stuff in order. You'd have 18 months to two years." And I thought, "Oh God. So what is it?" He said, "Well, it's IBM." And I'm pretty sure he didn't say definitively, but he said, "You need to go for a muscle biopsy and find out for sure." So, I said, "Well, where do I go?" He said, "Johns Hopkins is the place." And then I got my GP, Dr. David Allen to call the head there, Dr. Lisa Christopher Stein, and we have been best buddies for 10 years now. And she is wonderful. The treatment center there, the Myositis Clinic is... I've never seen so many passionate people who come to work and are so optimistic when it's so depressing. If you know what I mean.

Dr. Audrey Nath:
Sure. Yeah, absolutely. Yeah. It's a contradiction, isn't it?

Peter Frampton:
Yeah. They come to work every day, ready to fight it.

Dr. Audrey Nath:
Absolutely.

Peter Frampton:
So I'm very lucky. I'm on the second trial drug. I tried one, it didn't work for me, but I'm on a new trial drug right now. Been on it for two weeks.

Dr. Audrey Nath:
How are you doing?

Peter Frampton:
Well, no side effects or anything. Well, what they had noticed in a recent trial with this drug was that a few people, I don't know how many, it didn't regress. It won't regress, but it will stop or slow down.

Dr. Audrey Nath:
Fingers crossed.

Peter Frampton:
Exactly.

Dr. Audrey Nath:
That'd be awesome. Yeah.

Peter Frampton:
So yeah, I can't wait to tell people that it's working, obviously, otherwise we're onto the next, and that's why I started the fund, for testing. For trial drugs.

Dr. Audrey Nath:
Yeah, because you were in trials yourself. Right?

Peter Frampton:
Right.

Dr. Audrey Nath:
And you thought, "Hey, I can help with this."

Peter Frampton:
Yes.

Dr. Audrey Nath:
That's incredible. And have you been speaking with a lot of other patients with IBM as part of your foundation?

Peter Frampton:
I have. I speak with them, I catch up with them, and I met a lot of them on my farewell tour, and we would ask, "If you have IBM, please come back stage and Peter would love to meet you." So I did that and we'd leave them, and the last people in the meet and greet line, that's when you could have meet and greets.

Dr. Audrey Nath:
Oh, right.

Peter Frampton:
And so I would have time to talk with them, and it's kind of like a club, and I get excited talking to them because we're all fighting for the same thing and wishing for the same thing as well. And I see people in different stages, it can be little depressing obviously, because I know the end game, but if we can stop it along the way, please, that will be wonderful. And that's what we're fighting for.

Dr. Audrey Nath:
Give you some more years of guitar playing and touring.

Peter Frampton:
That's right.

Dr. Audrey Nath:
Your tour is still going right? There's some 2022 dates, yeah?

Peter Frampton:
I was with another doctor, I thought, "I'm going to get my throat checked." I haven't had any problems or anything, but basically I think I had acid reflux, and I was worried that with the big C, I wonder if I've got this. So I went to the doctor, and I got time to go to doctors now, and so he said, "You have the throat of a 40 year old." He said, "Your vocal chords are tight together." And I said, "Well, I do vocal exercises for 20, 30 years." He said, "You can tell." So he said, "Little acid reflux." I said, "Oh, is that what it is?" He said, "Yeah, that's what it is."

Dr. Audrey Nath:
Goodness.

Peter Frampton:
Anyway, but he said, "So what are you doing?" So I said, "Well, I finished my... we got shut down with the Farewell Tour, I wanted to go to Europe. And there's a possibility we can go to Europe in November, but I don't know if I'm going to be good enough to-" He said, "Go."

Dr. Audrey Nath:
Okay.

Peter Frampton:
He'd like that. And I was taken aback because I never met this guy before, but he said, "You know what? They want to see you."

Dr. Audrey Nath:
Absolutely.

Peter Frampton:
And I said, "Yeah, but I want to sound good." But he said, "You will. You'll make it."

Dr. Audrey Nath:
For sure.

Peter Frampton:
So it took this complete stranger to help me make my mind up, and life is so wonderful.

Dr. Audrey Nath:
It's serendipitous in that way. Isn't it?

Peter Frampton:
Yeah. So we're doing eight shows in UK in November. Hopefully, please keep working. The fingers.

Dr. Audrey Nath:
He's talking to his fingers.

Peter Frampton:
Sorry. Yeah, this isn't video.

Dr. Audrey Nath:
I'm curious. Have you been doing physical therapy? Has that helped you at all? What do you think?

Peter Frampton:
If I didn't do... I work out six days a week, I'm lucky enough I can afford to do that. I have two trainers.

Dr. Audrey Nath:
That's great.

Peter Frampton:
A weekend trainer and a week trainer, and they're both phenomenal. And they both work with Parkinson's patients.

Dr. Audrey Nath:
Okay.

Peter Frampton:
So I'm their first IBM, but there's obviously a lot in common with the muscles. Theirs is a completely different, I think it's-

Dr. Audrey Nath:
Different mechanism.

Peter Frampton:
Different. Isn't it-

Dr. Audrey Nath:
Because it's in the brain.

Peter Frampton:
Yes.

Dr. Audrey Nath:
Yeah.

Peter Frampton:
But the exercises are pretty much the same. You want to keep your core, you just want to keep everything exercised as often as possible because there might not be, I would like to tell you in a couple of months that we do have a cure.

Dr. Audrey Nath:
I know.

Peter Frampton:
But right now we don't, and the only thing we can do right now is exercise. It's incredible how it helps.

Dr. Audrey Nath:
And basically when you're on tour, do you think you'll make other accommodations? Do you think you'll sit more instead of jumping around kicking beach balls?

Peter Frampton:
No, I think that it's pretty much on the cause that I might have to sit down for a lot of the show.

Dr. Audrey Nath:
Yeah. Okay.

Peter Frampton:
Because unfortunately I get a little excited when I'm playing, and I'll forget and then I'll fall over.

Dr. Audrey Nath:
Oh, okay. So that'll force you to stay still-ish.

Peter Frampton:
Yeah, well, when we get into rehearsals, which will be a month before we go, obviously for at least two weeks, probably three weeks, so I can get the chops going with the band. Yeah, hopefully we'll find out whether I'm going to sit or stand.

Dr. Audrey Nath:
It's interesting that you said that when you're performing, you forget that you're in another zone entirely and can forget about this huge condition you have, and it's interesting because there's other patients that tell us that when they're in that kind of flow state, that they may entirely forget about a disability.

Peter Frampton:
Yes.

Dr. Audrey Nath:
If it's music, if it's painting, whatever it is, we encourage people to find that.

Peter Frampton:
Music saves.

Dr. Audrey Nath:
Oh, that is what your shirt says.

Peter Frampton:
Oh, I'm sorry, there's no video. It says, "Music saves."

Dr. Audrey Nath:
Yes. Amazing. Is there any other stuff that you do like meditation, or do you pretty much just pour yourself into your music to find that mindfulness?

Peter Frampton:
Yeah, basically the music, and I read something from one of my compadres with IBM, and they said, "The thing with IBM is if I do one thing every day I can have a great day." And that is after lunch, I take a nap.

Dr. Audrey Nath:
Oh.

Peter Frampton:
I sleep. I take a 45 minute to an hour nap, no more. And because it is tiring.

Dr. Audrey Nath:
Sure. Yeah, I can see that.

Peter Frampton:
Just obviously, walking is tiring, going upstairs is tiring.

Dr. Audrey Nath:
Oh, I bet.

Peter Frampton:
And luckily I'm moving to a house with no stairs.

Dr. Audrey Nath:
Ooh, there we go.

Peter Frampton:
Yeah.

Dr. Audrey Nath:
Seriously, it's something you don't think about until you have that weakness.

Peter Frampton:
I know.

Dr. Audrey Nath:
How much an impediment stairs can be. What does this Farewell Tour mean to you personally?

Peter Frampton:
Well, we did the American, Canadian portion of this and got through it right before in '19, right before COVID in '20. And it's a very strange feeling going out each night, knowing that at the end of the show you're going to say goodbye. Which I don't say goodbye. I just wave, and I say, "I'm not going to say goodbye." And what I want out of it is what the audience wants out of it. It's for them. It's not so much for me, even though I need to say goodbye, even though I don't say goodbye.

Dr. Audrey Nath:
Yeah, I see what you mean.

Peter Frampton:
But it's the audiences...I've never experienced the feeling that I got every night on the Farewell Tour here. It's a tear jerker, it really is, and I can't do that because it'll stop me being able to sing. You can't cry and sing, but they are over the moon supportive. My fans and those people, all the people that are... I looked at a tweet from somebody, and they said, "Yeah, I'm finally going to see Peter Frampton in the Albert Hall in November. I'm coming all the way from Melbourne, Australia."

Dr. Audrey Nath:
Oh my goodness. That's a flight.

Peter Frampton:
I know. It's-

Dr. Audrey Nath:
Oof.

Peter Frampton:
Thank you. I mean really?

Dr. Audrey Nath:
Yes!

Peter Frampton:
It's quite amazing, and I've read other ones of people coming halfway around the world to one of these European shows. So I can't thank everybody enough, but the more the merrier, and it's a fun show. There's nothing sad about the show. It's all upbeat.

Dr. Audrey Nath:
You'll just be sitting and not kicking the ball around. That's all.

Peter Frampton:
That's right.

Dr. Audrey Nath:
That's the only difference. That's it. I want to know from you, is there anything else you would like the audience to know about the Peter Frampton Myositis Research Fund, anything coming up?

Peter Frampton:
Every ticket in America and Canada tour went, a portion of it, I think a dollar, went straight to my foundation.

Dr. Audrey Nath:
Oh, the Farewell Tour tickets?

Peter Frampton:
Yes.

Dr. Audrey Nath:
Oh, cool.

Peter Frampton:
So we're hoping to be able to do that, the same thing over there.

Dr. Audrey Nath:
In Europe. Okay.

Peter Frampton:
Yes.

Dr. Audrey Nath:
Oh, that's great.

Peter Frampton:
Even if it means raising it $1 or whatever.

Dr. Audrey Nath:
Sure.

Peter Frampton:
I think people will understand. It's a great cause.

Dr. Audrey Nath:
That's awesome. You get to enjoy a show and contribute to research for another clinical trial. That might benefit you or someone else.

Peter Frampton:
Yes. We also had the Shriners came and sold one of the latest album, whatever it was at the time, as if they were going down the aisles, selling drinks, but they were selling the CDs, and 50% of that went to Shriners and 50% of that went to my foundation. So I didn't see it. We didn't see any money from those. It was perfect. And it made a huge difference to the-

Dr. Audrey Nath:
That's awesome.

Peter Frampton:
We raised just on the tour, it's above it now, but we raised over $300,000, so that was a great way to start.

Dr. Audrey Nath:
Yeah, for sure. It's incredible, the power that music has to bring people together, but then also to raise awareness for conditions like this that people may not have heard about. Thank you for using your voice that you could be using to talk about a lot of other things, to talk about myositis and new treatments.

Peter Frampton:
Obviously it's very near, and I shouldn't say dear to my heart.

Dr. Audrey Nath:
That's fair.

Peter Frampton:
But it's definitely something that I think about all the time. And the thing also is that there's other people, many other people out there, not as much as other diseases, we are what is known as a boutique disease, unfortunately, but there's many people out there that are finding out about it right now. And my hope is that I've already done something that will enable them to curtail or slow down or stop what they have.

Dr. Audrey Nath:
Absolutely, and possibly even people listening, thinking, "Oh, actually I have had this new weakness. I've been so busy. I haven't dealt with it." Maybe even for someone to seek care that might not have otherwise.

Peter Frampton:
Right.

Dr. Audrey Nath:
So, I just want to say thank you so much for sharing your time with us and your story. And yeah, if one person listening to this gets an idea that might help them, that's awesome. And I think it will be more than one person. Thank you so much for joining us.

Peter Frampton:
Let's hope that we have, next time we speak, we do have something to tell everybody that's just fantastic. Let's keep up-

Dr. Audrey Nath:
Yes, please come back.

Peter Frampton:
Fingers and toes crossed. Okay?

Dr. Audrey Nath:
Yes. I want to see how you do.

Peter Frampton:
If it happens, I'm coming back. Okay?

Dr. Audrey Nath:
Yes, we want to see how you do with this trial for sure.

Peter Frampton:
Exactly.

Dr. Audrey Nath:
Keep us updated. Absolutely. Thank you so much.

Peter Frampton:
All right. Thank you, Audrey.

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Dr. Audrey Nath:
If you don't live in the US, you can still enjoy Brain & Life online at brainandlife.org. To learn more about inclusion body myositis or IBM, we caught up with Steven Greenberg, MD, professor of neurology at Harvard Medical School, where he is a neuromuscular neurologist, who also is involved in numerous clinical trials. Welcome to the podcast, Dr. Greenberg.

Dr. Steven Greenberg:
Thank you very much, Audrey.

Dr. Audrey Nath:
First off, for our listeners, what is inclusion body myositis?

Dr. Steven Greenberg:
Inclusion body myositis is a skeletal muscle disease. It's a progressive disorder that largely affects several muscle groups in particular. Particularly muscles that control walking, the grip muscles as well, and the swallowing muscles. Although as the disease progresses, it can affect most of the skeletal musculature system.

Dr. Audrey Nath:
When you see patients, what is the first thing they usually notice? And how old are they?

Dr. Steven Greenberg:
Yeah, most patients are over the age of 50 when they develop symptoms. Typically, it's in the early 60s for most patients. The typical symptoms that they complain about are difficulty with walking, falling, collapsing of the knee, particularly from quadriceps weakness. Many of them have difficulties with grip and hand grip and complain of difficulty holding things, opening jars, turning keys, and a smaller number, principally complain of swallowing difficulties.

Dr. Audrey Nath:
Peter Frampton was mentioning that in the beginning of his course, he noticed he was having trouble hiking with his son, so with some lower extremity weakness and that he was starting to have some falls on stage that he had never had happened before. So that sounds it's consistent with what you've seen with other patients as well, that they can start out with some weakness. How is inclusion body myositis similar or different from other muscle diseases?

Dr. Steven Greenberg:
Well, there's quite a big range of muscle diseases as you could imagine. Often we think of the inherited muscle diseases versus the acquired ones as a branch point. They have very different ages of onsets. This one certainly is distinguished by a later age of onset. We're not talking about children or adolescents or people in their 20s or 30s at all. So that's usually one clue to narrow it down. This one is also very slowly progressive, and it's particularly the muscle groups that it affects, which are a little distinguished from most other forms of acquired muscle diseases. So most other acquired muscle diseases are very proximal, which means that you'll have difficulty with your shoulders, raising your arms up over your head,, for example. Difficulty bending your legs at the hip for example. This one tends to just be a different group of muscles, so people won't complain of trouble raising their arms over their head. They'll complain with grip, their hand involvement, for example. So that's typically-

Dr. Audrey Nath:
Or playing guitar.

Dr. Steven Greenberg:
Or playing guitar, of course. I haven't met Peter, but I would've imagined if that would be a symptom that would be quite impairing.

Dr. Audrey Nath:
To help us understand a little more about muscle disease, many of our patients, patients that I've seen in the past have had some weakness and especially if they're in the older age group, the first question is, "Doc, do I have ALS?" is sometimes the first question that comes out even before the history and before the physical. Can you help explain what you might see as differences in clinical presentation between ALS and an entity like IBM?

Dr. Steven Greenberg:
Well, sure. A lot of symptoms may be superficially related, but as a neurologist our first task is to decide where in the nervous system the problem is. So multiple sclerosis is a disease of the central nervous system. ALS is a combination of central nervous system neurons and peripheral nervous system neurons. Muscle diseases like IBM are purely out in the periphery. And so they can all produce similar symptoms like I have trouble walking or I have trouble using my arms, but physical examination, sometimes even the nature of the symptoms can help to distinguish between whether we're dealing with a brain or spinal cord disease versus a peripheral nerve disease versus a muscle disease. And if it's still confusing after that, which it rarely is, usually it's pretty clear from history of physical exam, but if it's still confusing, then electro diagnostic testing with EMG can be quite decisive in distinguishing those three possibilities.

Dr. Audrey Nath:
So EMG, or electromyography, and nerve conduction studies are what they say in neurology residency, right? It's an extension of our physical exam. Since we have some listeners who may be undergoing workup for these diseases, can you briefly tell us about what is involved in an EMG and a nerve conduction studies?

Dr. Steven Greenberg:
An EMG test involves roughly two parts. One involves stimulating nerves with little bits of electrical current and measuring the responses. The second part involves putting needles into muscles and recording electrical activity in muscles. And the test has some strengths and weaknesses. It's used throughout neuromuscular disease for different types of problems. In the setting of say IBM, for example, it's extremely helpful to be able to decide why a particular muscle is weak, whether it's weak from a muscle problem or a nerve problem.

Dr. Audrey Nath:
That makes sense. When you have patients with inclusion body myositis and the diagnosis is made possibly with these studies, and then you sit down and tell them about it. What do you tell them about treatment or therapies and what they should do moving forwards?

Dr. Steven Greenberg:
Yeah, you always try and give people the best possible information to make their own decisions. So certainly no value in trying to hold back and worry about people's anxiety and so forth. I think people are best served by really just telling them everything you know, everything they want to hear about a disease. So for IBM, there are things which are very clear. It's very clear that there's no reliable responses to any medications that we currently have. That most patients progress with the disease. We certainly have numbers in terms of average times to when people need to use something, to help them with walking, for example.

Dr. Steven Greenberg:
Most patients have already had symptoms for several years, by the time they first get diagnosed. So they're already into this, and so just going through the facts here, I think there are examples of patients who have done extremely well. Just about everybody, and published series, there's always a couple of patients who 15 years into their disease have barely progressed. And so it's always nice to point that out to people that we don't have called a crystal ball, and people can progress at different rates and some people seem to be very slow. Theirs are faster. And to provide that information.

Dr. Audrey Nath:
You mentioned that sometimes when you see people it's actually been several years probably since their symptoms started. What do you think is happening? Do you think people are chalking up the weakness to something else or thinking it's a sports' injury? What do you think it is?

Dr. Steven Greenberg:
Well, one is the very slow progression. So people are not generally different month to month. For example, even over six months, even over a year, it can be difficult for some people to look back and say what's different. The other thing is it certainly does tend to happen in an older age group. Mean age is 65 for this, and people may attribute some of the mild symptoms they've been developing to age.

Dr. Audrey Nath:
I see.

Dr. Steven Greenberg:
So that's half of it. The other half is on the physician end, too. It's not a diagnosis that's often made very easily by physicians, certainly not by general practitioners.

Dr. Audrey Nath:
Sure.

Dr. Steven Greenberg:
Patients often move on to general neurologists or rheumatologists at that point. It can be a little subtle to make the diagnosis early on.

Dr. Audrey Nath:
This particular condition, based on what I've read about it, doesn't appear to really run in families, but some muscle diseases do. How do you help distinguish those things or do you just know, make the diagnosis and then think, "Ah, this one tends to be genetic and possibly do genetic testing or checking other family members?"

Dr. Steven Greenberg:
Well, this particular disease has such a characteristic presentation. If I'm not 95% sure from history and physical examination, that would be unusual for this disease. So I'm pretty certain about what this is after it's simply a history and a physical examination of a patient. There's almost nothing else it can be if the physical examination is very characteristic. So we don't usually get into issues of genetic testing. As you say, this is not a genetic disease at all. It is an autoimmune disease and very rarely we run into patients who have a family member who's affected. As is the case for almost all autoimmune diseases. Those risks have to do with MHC haplotypes that get carried and put people at risk for autoimmune disease. So you see that, that increased risk throughout autoimmune diseases and as for here.

Dr. Audrey Nath:
Do any of the patients tend to have other autoimmune diseases or not really?

Dr. Steven Greenberg:
Yes. So, something like 15 to 20% have Sjogren's syndrome, that's the most associated disease, which also is quite a refractory autoimmune disease.

Dr. Audrey Nath:
Can you tell us a little bit about Sjogren's syndrome just for our listeners who might not know what that is?

Dr. Steven Greenberg:
Sure, Sjogren's syndrome is an autoimmune attack on salivary and lacrimal glands on the glandular cells that produce tears for the eyes and saliva in the mouth. And so the main manifestations are dry eyes and dry mouth, but also the clinical consequences of that. So if you have dry eyes, you have problems with your cornea. Now that can be quite substantial if you can't make saliva, you actually develop a lot of problems with your teeth over the protective benefits of that. Then it can also, Sjogren's also affects other organs as well.

Dr. Audrey Nath:
Wow. So then people can actually have both of these. That's interesting.

Dr. Steven Greenberg:
Yeah, rheumatoid arthritis also travels in this population to a smaller degree. So there's a handful of autoimmune diseases that people can develop with this, but those are the main two.

Dr. Audrey Nath:
Do you end up working with rheumatologists for some of these cases?

Dr. Steven Greenberg:
Many patients with IBM see one or the other. Rheumatologists are quite comfortable with this diagnosis and caring for these patients. So if there is a specialist that's involved outside of primary care, internal medicine, it would be a rheumatologist or a neurologist who sees this patient.

Dr. Audrey Nath:
I think there's people out there that might not know, but we often have to work together really closely with other specialties and speak each other's languages and work together as groups. I think this is one example where it's definitely multiple specialties that can be working together. We're here to help people from different specialties, primary care, neurologists, rheumatologists, all sorts of different people are here to think through these issues and help try to find out the diagnosis. That's why we're all here. Well, thank you so much for joining us today, Dr. Greenberg.

Dr. Steven Greenberg:
Oh, sure. It's been my pleasure.

Dr. Daniel Correa:
Thank you for joining us today on the Brain & Life Podcast. Follow and subscribe to this podcast, so you don't miss our weekly episode. You can also sign up to receive the Brain and Life Magazine for free at brainandlife.org.

Dr. Audrey Nath:
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Dr. Daniel Correa:
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Dr. Audrey Nath:
Special thanks to the Brain and Life team, including...

Dr. Daniel Correa:
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Dr. Audrey Nath:
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Dr. Daniel Correa:
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Dr. Audrey Nath:
We are your hosts.

Dr. Daniel Correa:
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Dr. Audrey Nath:
And Audrey Nath, beaming in from Texas and on Twitter @AudreyNathMDPhD.

Dr. Daniel Correa:
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Peter Frampton on Rockin’ for Research to Cure Inclusion Body Myositis

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