Understanding and Managing Frontotemporal Dementia

Frontotemporal dementia (FTD) is a type of dementia caused by degeneration in the temporal and frontal lobes of the brain. Our guest discusses the symptoms, risk factors, prevalence, and course of the disease, along with information on how a doctor diagnoses FTD and what families and caregivers can do after diagnosis. Guest: Howard “Howie” Rosen, MD, behavioral neurologist at the University of California San Francisco Memory and Aging Center

Episode Topics:

A definition of Frontotemporal Dementia (FTD) 1:02

How common is FTD? 3:53

Genetic risk factors of FTD: 4:30

Non-genetic risk factors of FTD: 6:40

Symptoms of FTD: 8:40

How a clinician diagnoses FTD: 14:03

What is the course of the disease? 16:20

What to do after a diagnosis: 18:51

Family and caregiver experience of FTD: 22:36

Community support and networking: 25:53

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Howie Rosen, MD
Howie Rosen, MD

Show notes

During the interview, Dr. Rosen mentioned two groups that offer support for people with FTD and their caregivers. They are the Association for Frontotemporal Degeneration and the Alzheimer's Association

Learn more about Frontotemporal Disorders at the National Institute on Aging website


NATHANIEL CHIN: I'm Dr. Nathaniel Chin and you're listening to Dementia Matters, a podcast about Alzheimer's disease. Dementia Matters is a production of the Wisconsin Alzheimer's Disease Research Center. Our goal is to educate listeners on the latest news on Alzheimer's disease research and caregiver strategies. Thanks for joining us.

NATHANIEL CHIN: My guest today in Dementia Matters is Dr. Howie Rosen, a behavioral neurologist at the University of California San Francisco Memory and Aging Center. He evaluates new patients in the memory clinic and provides continued care for some of them in the continuity clinic. Dr. Rosen's primary research interest is in frontotemporal dementia, particularly the disease’s effects on emotional symptoms. Dr. Rosen is also a teacher to medical students, residents and fellows. Welcome, Dr. Rosen, to Dementia Matters.

HOWIE ROSEN: Thanks very much Nate. It's a pleasure to be here on this snowy day in Madison, Wisconsin.

NATHANIEL CHIN: On today's program, I want to focus on frontotemporal dementia, a condition that is less common than Alzheimer's disease, but equally important to address in clinic and research. To begin, can you explain to us what is frontotemporal dementia and how is it different than Alzheimer's disease?

HOWIE ROSEN: Frontotemporal dementia is a neurodegenerative disease like Alzheimer's disease. What they have in common is that the nerve cells, the neurons, are shrinking. Some of the nerve cells are even dying, and that's happening in frontotemporal dementia, similarly to Alzheimer's disease. What's different about frontotemporal dementia than Alzheimer's disease is that in Alzheimer's disease, there are specific proteins that have been identified that cause the problem: tau and A-beta. I'm sure you've talked about those in past podcasts. In frontotemporal dementia, the proteins that are believed to cause the trouble are usually tau, but a form of tau that's a little bit different than the Alzheimer's tau, and this other protein called TDP 43. So, one way that frontotemporal dementia is different than Alzheimer's is which proteins we think are causing the problem. There are other rare varieties of frontotemporal dementia that are also caused by this protein called fused in sarcoma or FUS. So one way they're different is which proteins caused the problem. 

The other way they're different is what regions of the brain are affected. In Alzheimer's disease, the regions that are most commonly affected are the hippocampus and nearby structures that are important for memory. That’s why memory is usually one of the earliest symptoms in Alzheimer's disease. But in frontotemporal degeneration, the disease can either affect specific parts of the frontal lobes that are important for emotions and social behavior, or parts of the temporal or frontal lobes that relate to language and communication. In frontotemporal dementia, the early symptoms are often features of what we call aphasia, which is an acquired disorder of language, or changes in social and emotional functions and that's the main problem that manifests early.

NATHANIEL CHIN: Is it fair to say that the similarity would be this end result of brain cell death and in essence dementia, but some of the key differences: it's a different protein, the region of the brain that's affected is different in the beginning, and then therefore the symptoms a person experiences is different.

HOWIE ROSEN: Exactly. Right.

NATHANIEL CHIN: In people under the age of 65 who have brain changes, how common is frontotemporal dementia?

HOWIE ROSEN: It’s important to point out that dementia in general is much less common in people under the age of 65 than over the age of 65. But I'd say probably around 10 to 20% of dementia might occur in that age group [under the age of 65]. In that age group of the younger than 65, it's believed that frontotemporal degeneration could affect half.

NATHANIEL CHIN: Oh wow. So, this is not an uncommon thing for people. 


NATHANIEL CHIN: Is there a genetic component to frontotemporal dementia?

HOWIE ROSEN: There is. It’s important to point out that genetics is complicated, needless to say. Sometimes we talk about it in ways that are too simple, even for the lay person. I always point out that everything about us has a genetic component. Our eyes, our hair color, our height, lots of other things. But a lot of those genetic effects are tiny little effects that can balance each other. You might have one gene that maybe makes you likely to be a little taller or a little heavier, and another gene that might make you more likely to be a little shorter or a little thinner. Similar with your metabolism and lots of other things about us. Now in that sense, both frontotemporal dementia and Alzheimer's and probably every other disease we can think of has genetic components. A lot of times when we talk about genetics, that's what we researchers are studying.

There's another kind of genetic risk that we often think of when we're talking about genetics, which is a particularly abnormal change in a gene that is so problematic that it means that the disease is very likely to occur. We call that kind of abnormality a mutation. Mutations can be a cause of both Alzheimer's and frontotemporal dementia. But in Alzheimer's disease, it's a pretty rare cause. Probably about one or 2% of Alzheimer's diseases are due to that kind of genetic abnormality, a mutation. In frontotemporal dementia it's probably a higher proportion, maybe 10 to 20%. That’s a long answer to your question. The short answer being yes.

NATHANIEL CHIN: I appreciate what you're saying that when it comes to genetics, it's not just the genes, it's the interactions of our genes. I haven't heard that said before, so I think that's a really good point. Now we're talking in our podcast about risk factors for Alzheimer's disease. Are the risk factors not genetic for frontotemporal dementia?

HOWIE ROSEN: One way to answer that is a lot of the risk factors that we talk about in Alzheimer's are probably risk factors for every dementia. The problem is it's not as easy to prove it because these risk factors have relatively small effects that are only visible if you get large numbers of patients, hundreds to thousands, to tens of thousands. That's where we know most of what we know from Alzheimer's, looking at data like that. It's not easy to get thousands of patients with frontotemporal degeneration to demonstrate that these effects might be the case. We have some recent findings from a study we did that hopefully will be published later this year or early next year that look at people with a genetic form of frontotemporal degeneration. We looked at reading habits and exercise habits; both of those have been shown to be behaviors that influenced the likelihood of developing Alzheimer's down the road. It turns out that they seem to influence the rate of progression in these mutation carriers with frontotemporal dementia as well. That’s one example and we're seeing similar findings from other studies of genetic frontotemporal degeneration. That’s some evidence of what I said earlier, which is a lot of those Alzheimer's risk factors may apply much more generally.

NATHANIEL CHIN: Fortunately, recommending exercise and cognitive engagement and healthy foods seems to benefit more than just one thing.

HOWIE ROSEN: I'm never going to feel bad about recommending those.

NATHANIEL CHIN: What is the most common symptom of someone who comes into the clinic and you end up diagnosing with frontotemporal dementia?

HOWIE ROSEN: About half of the time it's these changes in social and emotional functions. Those would be things like loss of social decorum. The easiest way that I ask about that in a clinic is I'll ask people about their family member and we'll talk about the fact that it's hard to ask the person themselves. If you can ask their family member, has your father, a husband, a mother, whoever, forgotten how to behave in public? I think that allows people to picture the kinds of symptoms we're talking about. And then I'll give examples like eating off other people's plates at restaurants or talking about people that you don't know in ways that you ought not to do, like calling people fat out loud at a mall or things like that. And the thing is, all our lives are a little different. There are too many examples to come up with one that fits everybody. But those kinds of examples usually trigger people to start thinking of ‘Well, yeah, they did do that’. So it's those kinds of symptoms. 

Loss of empathy often looks a little different. That's another one we see a lot where people seem to not care about people as much as they used to. That usually most manifests when something happens that requires you to step up your emotional support and energy. Somebody might have an injury or go to surgery and the person with the disorder doesn't respond in the way that their loved one would expect that they would have in the past. So, I might give an example of we've had patients who came home from gallbladder surgery and that night their husband is asking them, ‘Why don't you go cook dinner, you cook dinner every night, what's going on?’ They wouldn't offer to bring them food in bed or other things to help them recover from the injury. Things like that. Sometimes if no events come up like that where it's obvious to the family that a reaction should have happened and it didn't, it's not so easy to detect this. You have to really know to ask for it. Depending on what's going on the last few years in different people's lives, it might be easier or harder to pick it up. That’s the social and emotional thing. I have to point out that's about half of frontotemporal degeneration and the other half is these language disorders.

One of the more common symptoms is profound loss of the ability to use words and recognize words. It's a normal phenomenon as we age that we all have a little more trouble retrieving the word we want. I don't want to say that everybody with that problem has frontotemporal dementia, far from it, but if it becomes bad enough that it interferes significantly with communication, that could be the sign of a degenerative disease. It could be Alzheimer's disease too. But in frontotemporal dementia that could be a profound symptom. One of the unique things about frontotemporal dementia is in that situation, you could sometimes not even recognize the word. So, if I can't think of a word and I'm grasping for it and you realize I'm trying to say the word – ‘this thing that's picking up my voice, what's it called? Oh yeah, right. A microphone. Yeah, that's the word’. If I do that, and you say microphone, I recognize that you've said the correct word. And I say that's the one I want. So, there's no problem with me knowing what the word is and what I'm supposed to do with it. I just need a… I couldn't come up with it on my own. But in frontotemporal degeneration they might say ‘Microphone? Maybe, I'm not sure’. In fact, they might even do that when people say the words not in that context, they might say ‘Oven? Oven. I should know what that is. What is that again?’ Oven is a very simple word but it would be more likely with the words that we use less commonly, but that that person should have known. I had one example of a patient who they were watching television and the term circumcision came up on the television and he said ‘Circumcision. Now what's that again?’ This would be a very rare symptom in Alzheimer's. I really never heard of it because of what regions of the brain can be affected in Alzheimer's, but it can happen in frontotemporal dementia. The last thing, another common kind of a language symptom, which is different than the one I just described, is a hesitancy, stumbling kind of difficulty with speech where you can't get your words out and you trip over the syllables. It's very difficult to enunciate essentially, your speech gets broken up, and well broken up into pieces and kind of robotic in some cases because of that nature and that's yet another form of frontotemporal generation.

NATHANIEL CHIN: While these symptoms are different than Alzheimer's disease, you as you said, there's some overlap and sometimes atypical Alzheimer's disease can look like this. So how do you diagnose frontotemporal dementia?

HOWIE ROSEN: The core approach is careful questioning. I even gave some hints in talking about this. I've developed and I think each clinician who gets experienced with this develops their own kind of a toolkit of questions they like to ask, which are meant to try to bring out if some of those symptoms are there or not. Really it's a very knowledgeable way of looking at what questions to ask. Also, hearing the answers many times over and over again, you start to know which ones sound like they need more follow up and which not. Essentially that kind of questioning is the main way to get there, so it therefore relies on a very committed and reliable collateral informant, a family member or close friend who can tell about the changes that have happened over time. 

In addition, we do use other testing to help reassure ourselves if we've made this diagnosis that the additional evidence supports it. Neuropsychological testing is a common tool in our business and that can document that certain kinds of reasoning and problem solving abilities are impaired and we would see that too. So, the neuropsychological testing can be helpful. Then brain imaging can be helpful in that there is a typical pattern of brain atrophy or volume loss that we can recognize even visually looking at the MRI. But those images can sometimes be misleading. Similarly, these neuropsychological tests can be abnormal for other reasons or not abnormal, even though somebody has frontotemporal dementia. They have to only be used as extra evidence when you feel like you have a history that points in that direction.

NATHANIEL CHIN: So similar to Alzheimer's disease, there is no definitive one task, one blood test or scan that can tell you it's frontotemporal disease.

HOWIE ROSEN: That's correct.

NATHANIEL CHIN: A common question that I get in clinic about Alzheimer's disease is, what does this course look like? I will often tell people Alzheimer's disease is progressive, but it's gradual. You're going to see different symptoms. But overall it's one of decline. Is that similar for frontotemporal or is there a different course?

HOWIE ROSEN: No, it's very similar in that sense that you mentioned it's gradual. Over months to years is when you'll notice change rather than weeks to days. I often make the additional point, even in Alzheimer's disease, that that rate of change isn’t constant for an individual either. Somebody might have a slow and insidious change over the last couple of years, but then they have a bad year where their change seems to be a little faster and after that they might plateau and slow down a bit. Or sometimes if everything's faster, everything really is faster after that. I tell people that because it's natural for us to want to extrapolate from what we've seen to what we think will happen in the future, or to assume that being at a certain stage means that you have X number of years left. I think those rules don't apply very well in any of these neurodegenerative diseases. There are even a lot of physicians who talk about them as if they do apply and that really isn't often that helpful for patients. I think you have to be pretty open-ended about when you expect something to happen. Although we can't pretend, we shouldn't expect that things will happen. One thing I'll say about frontotemporal dementia is that on average, unfortunately the course is faster than Alzheimer's disease; some studies have shown twice as fast. There's another phenomenon that happens in frontotemporal degeneration, which is that you can as an additional feature get amyotrophic lateral sclerosis or A-L-S, which is also sometimes called Lou Gehrig's disease, which is a disease of the muscle, the nerves that supply the muscles. That's a very debilitating disease that usually causes physical dysfunction and a lot of disability very quickly. About 10 to 15% of frontotemporal degeneration cases are also associated with ALS developing. In those cases, unfortunately, the course can be very fast, maybe only a couple of years from the development of the ALS symptoms to dying.

NATHANIEL CHIN: Unlike Alzheimer's disease, there are no specific medication treatments for FTD, no FDA-approved ones. I wonder, what can people do once they've been diagnosed or what do you recommend in your clinic?

HOWIE ROSEN: Even in Alzheimer's disease, although there are treatments, many of the problems that we have to face are not very well addressed with medication. In Alzheimer's, there are many social and emotional problems that are different than the kind we see in frontotemporal dementia, but they require us to take a different approach. In FTD, those are the core features. What I mean by different approach is to try to treat behavioral problems and other problems of social and emotional function, a lot of it is about understanding the person's environment, trying to teach the family how to adjust their responses so that they don't exacerbate the person's emotional trouble. A lot of us we have natural responses like, ‘Don't you remember that?’ And of course, in Alzheimer's disease, they don't remember that. We have to teach ourselves to not say that. Similarly we have lots of other natural ways that we interact with each other would serve us very well when all of our brains are functioning normally but have to be adjusted. Family and friend education is part of it. Sometimes moving or changing the environment to lessen the problem is part of it. A lot of that comes with expertise. Finding access to a clinic or a group that has a lot of experience in this kind of thing can help those things get implemented a little more effectively. Like all treatments of that kind of thing, it's never going to be 100% perfect. I usually tell families that if you get a 50% improvement in behavioral trouble, that's a great outcome and it's very important to set our expectations reasonably. 

I think it's important that there are some treatments that don't get used enough that might have value. In the setting of a disease that we know will deteriorate over time, traditional therapies like speech therapy and occupational therapy often are not applied out of this kind of pessimism that the disease is just going to work past this and why should we bother? But I think they can be helpful. I often say we have to press our colleagues who are in that area to go ahead and do the work anyway. Of course, some people are naturally wanting to do it, but others may need a little pressure, but that could help. There are some experimental approaches to speech and language therapy that are being looked at now that appear to be promising. There are some other kinds of therapies. I don't know if you've ever talked about a therapy called a magnetic stimulation or there's a kind of therapy called transcranial magnetic stimulation where we apply very small, basically magnetic pulses to the brain to kind of change the electrical current in the brain transiently. That therapy is used in depression sometimes and in some neurological settings. It's still being developed, but they're trying it in aphasias and it looks like it might be promising. There might be some papers about that in the next year or so. Of course, we're working hard to try to develop drugs for these diseases that would actually clean up these proteins or address the damage they're causing in some other way.

NATHANIEL CHIN: I'm so glad you mentioned family though, because even with Alzheimer's disease, this is a disease that affects more than one person. Being able to train and educate family members is a really important thing. With frontotemporal dementia or degeneration affecting different parts of the brain, there are probably unique experiences that caregivers and family members have with this disease, particularly. What kinds of things do you see in clinic?

HOWIE ROSEN: Another question we've learned to ask is, has the person's personality changed? That often is the right entry into the family explaining symptoms that help us make a diagnosis. That's not a question where you get a "yes" very often in Alzheimer's disease. I think that's an interesting question itself - what is our personality? The average person doesn't think of having a good memory or a bad memory as part of our personality, it's more about how we behave toward each other. Even as memory declines, a lot of people wouldn't say they're a different person. But in frontotemporal dementia, that's quite an early phenomenon with the social and emotional variety- the family member feels like a different person and that puts an extra burden on their family to be sympathetic to their problem and to care for them. If somebody with Alzheimer's is suffering and you want to help them they often show appreciation in some way or another. But a lot of times, for example, frontotemporal dementia patients may not. So, it's more hurtful and difficult to stick with it. 

Often, there are many social and emotional things that happen before the diagnosis. I don't know if you're gonna ask me about this, but frontotemporal dementia, if it sounds hard to diagnose, I'll tell you it is. Part of the reason it is, is because many, many physicians recognize that declining memory could be a sign of Alzheimer's disease. But when we hear about social and emotional changes, most clinicians don't think of a neurodegenerative disease. They think of depression or other psychiatric illnesses or midlife crisis. Somebody goes out and spends too much money, that's usually a psychiatric illness or them being bored and midlife. And often that's true, but it can be a sign of a disease in these cases. So all that's gone on. We’ve had families, couples that are pretty much on the verge of divorce by the time they get to us because this disease has decimated their relationship so much. Sometimes the fact that we can diagnose and say ‘It's not them, it's this disease’ can help. But sometimes all the emotional toll that this disease has taken, even that's not always enough, but it's hugely helpful to kind of reframe things for a family and say, ‘You know, it's not that they stop loving you, it's that this disease has really taken their ability to show it and to respond to you the way they would have’.

NATHANIEL CHIN: In Alzheimer's disease, we have a very strong community network and support system through our community organizations and memory cafes and things like that. Is there something specific for people with frontotemporal dementia?

HOWIE ROSEN: I think the leaders in helping us as a community develop that network for Alzheimer's disease is the Alzheimer's Association. I think the Alzheimer's association has an awareness of these other dementias. Somebody pointed out to me that the name of the Alzheimer's Association used to be Alzheimer's and Related Dementias Association, but it was harder to say. So, they got rid of that. My point is they are aware of it and I think they can try to connect one to resources. More importantly I want to point out there is a special organization called the Association for Frontotemporal Degeneration based in Philadelphia, they have a website and they're very committed to trying to connect people with resources to help them. Because this is a less common disorder, sometimes those resources are might be a collection of people far away, but who might get together on a phone meeting every month or a web based support group and things like that. Certainly in many communities there's a specialty center like this one that knows a lot about all kinds of degenerative diseases. They might have special resources for more unusual situations like this.

NATHANIEL CHIN: With that, I would like to end Dementia Matters and welcome you back again in the future. 

HOWIE ROSEN: Thanks very much.

NATHANIEL CHIN: Dementia Matters is brought to you by the Wisconsin Alzheimer's Disease Research Center. The Wisconsin Alzheimer's Disease Research Center combines academic, clinical, and research expertise from the University of Wisconsin School of Medicine and Public Health, and the Geriatric Research Education and Clinical Center of the William S. Middleton Memorial Veterans Hospital in Madison, Wisconsin. It receives funding from private, university, state, and national sources, including a grant from the National Institutes of Health for Alzheimer's Disease Centers. This episode was produced by Rebecca Wasieleski, and edited by Bashir Aden. Our musical jingle is Cases to Rest by Blue Dot Sessions. Check out our website at adrc.wisc.edu. You can also follow us on Twitter and Facebook. If you have any questions or comments, email us at dementiamatters@medicine.wisc.edu. Thanks for listening.