Lewy Body Dementia with Dr. Trey Bateman

[Dr. Mia]: 0:39

Welcome to Ask Dr. Mia podcast. Today I have a special guest with me, Dr. Trey Bateman, who is a behavioral neurologist that I work with. And Trey and I have shared a number of patients together and wanted to invite him to the podcast to talk a little bit more about Lewy body dementia and other non-alzheimer's type of dementias because he is certainly the colleague that I turn to. most commonly when I have questions about those two types of dementia subtypes. So Trey, tell us a little bit about yourself and how you got interested in behavioral neurology.

[Trey Bateman]: 1:22

Sure, thank you for having me on Mia. Really appreciate the opportunity to talk about this. So I got interested in behavioral neurology really early on. I was sort of the weird person who went to medical school saying, oh, I want to be a behavioral neurologist, which is kind of an odd thing to go to medical school wanting to be. When I went to college in Greensboro, North Carolina, I was sort of back and forth between biology and psychology as a major and distinctly remember reading in the first chapter of a biological psychology textbook, you know, the chapters that you're never tested on, that there's sort of nothing terribly relevant that you need to see there, but I remember loving biological psychology and thinking, well, how can I do stuff with this? And it mentioned, you know, going to medical school and becoming a physician. And I thought, well, sure that seems like a good idea. And it was really sort of as simple as that for getting me started. And when I ended up going to medical school in Chapel Hill, I met a behavioral neurologist there who specialized in dementia and just fell in love with the idea that understanding the structure and the connectivity and the function of the brain could help you understand behaviors and emotions and how we interact with the world. And so, you know, for me, I just wanted to find a way that I could kind of think about that every day because it was so exciting and interesting to me. And behavioral neurology was the subspecialty that allowed me to do that. Now, I sort of vacillated a bit between psychiatry and neurology and I think I would have been happy doing either. The subspecialty that I trained in is formally called behavioral neurology and neuropsychiatry. So you can do the same fellowship from either direction. And I think I would have probably been happy and fine doing it from either, but I ended up training as a neurologist first and then going into behavioral neurology from there.

[Dr. Mia]: 3:43

Got it. Yeah. And I think that correlates a little bit with my interests. I was definitely the late differentiator. Didn't know what I wanted to do from, for many years, but definitely enjoyed internal medicine, older adults, geriatrics, psychiatry, uh, and, and neurology. I think what was a little interesting about neurology, at least in residency training, so much of it is focused on localizing the lesion as well as stroke management, seizure management, that there really isn't as much correlation with the emotion behavior aspect that shares similarities with psychiatry. For me, I know some of my earliest inspirations for working with patients living with dementia and their families were through house calls. I remember taking care of a... a family who lived in Greek town in Baltimore, and I would go down the steps in the row house to go see their father who had dementia with Parkinsonism symptoms, and he really had a lot of mobility difficulty, and just the kind of extraordinary sacrifices and rallying of the whole family around him. I think they ended up giving me a card that said, I'm their honorary Greek daughter,

[Trey Bateman]: 5:12

Hehehehe

[Dr. Mia]: 5:12

which really made me feel honored. I'm just curious if any patients or any stories like that, that kind of inspired you along with the book.

[Trey Bateman]: 5:23

Yeah, so in medical school, as a third-year medical student, you know, and I echo completely that point about neurology, you know, so much of the training takes you away from those things that I was most drawn to and most interested in. I happen to also, I love the idea of localization and sort of understanding where things are going wrong in the brain. And what I, the things that I remember a lot about my early experiences of taking care of patients with my mentor, Dan Kaufer, who was at UNC, is that he would often see people that had unusual presentations. Things that didn't fit into, when we think of dementia as a syndrome, most people, including most doctors, especially those who don't spend all of their days taking care of... dementias have kind of this caricature or set of caricatures about what those symptoms ought to look like. And he would see people that would come in, you know, in their 50s who had profound behavior changes, losing their jobs, they've, you know, they're doing things that are so out of character and they would have, you know, they were told, well, you just, you know, they're that's just them, they're having a midlife crisis or something like that. And it turned out that they had maybe behavioral variant front or temporal dementia. Or they would come in psychotic with hallucinations and delusions. And, you know, they turned out to have Lewy body dementia. The patient that I have that really remind sort of is in my mind for. The reason that I do what I do is when I was a third year medical student, the first patient that I took care of on a neurology rotation was a woman who had been transferred in from an outside hospital in her mid-50s with rapidly progressive neurological symptoms. And her cognition was actually pretty good when we first saw her. And so she predominantly had a rapidly progressive cerebellar ataxia. So, discoordination with myoclonus, so twitching, jumping muscles, and some mild cognitive impairment. And pretty quickly, on imaging and a lumbar puncture, we determined that she had Creutzfeld-Jakob disease. It's a prion disease and a rapidly progressive dementia that's universally fatal. And I was able to take care of her. in the inpatient setting and I was actually the one who after the diagnosis was delivered went in and talked to her about her symptoms, her life, and ended up doing the describing the National Prion Disease Surveillance Center autopsy program to her. And I was struck by how much it meant to her to be able to do that because it was the only thing she had control over. she had been given a horrible disease that if you've never seen, it's kind of hard to even imagine how relentless and terrible and quick it is. And the ability to give her that diagnosis and the power, someone's giving her her agency back to say, you know what, yes, this is happening, but I can at least give an answer to the people that I love. And so I did the, helped her arrange that. that really stuck with me, I think, partly because it was a diagnosis that kept getting missed, you know, when she would go to the ER in the weeks prior to that, when she would see her outside doctors. And a lot of the patients that I end up seeing are folks that because they're unusual, I'm rarely the first person seeing them. And I'm often asked to provide second, third, or fourth opinions for some of these folks, which is really common for people with these atypical or less usual forms of dementia.

[Dr. Mia]: 9:45

Yeah, absolutely. And I think I share that similar similar experience of being able to give a diagnosis that accurately describes what they have been going through. And I think it is very empowering to know even if the knowledge of the disease is terrible, and it's not something that gets better knowing what it is and that they're reassuring them that they're not crazy, they're not making up something, this is not just depression, this is not just in their brain. I think that is very powerful. And that gets to kind of one key feature of Lewy body dementia that I think as learners and trainees that I work with really have a difficult time kind of translating what the textbook definition is to what patients come in presenting at. So for, I'll ask you in just a minute to kind of highlight some of the key features of Lewy body. But the one I wanted to talk about is what's called fluctuating mentation, which really sounds kind of benign or not that serious, but different people now that I've treated a number of patients with Lewy body dementia, they really describe them in a series of different ways. I think if you're not the diagnosis, it could really present like UTI or a stroke or a TIA. Maybe it's a seizure. What is going on? You just like zoned out.

[Trey Bateman]: 11:21

Thanks for

[Dr. Mia]: 11:22

So,

[Trey Bateman]: 11:22

watching!

[Dr. Mia]: 11:22

um, I will try if you can tell the listeners who maybe older adults who have really never understood what Lewy body or maybe even some of the other clinicians listening, what, how do you, what are some key features of Lewy body?

[Trey Bateman]: 11:38

Yeah, so I'll talk about just each of those key features and then I can give a more elaborated description of fluctuating cognition. So, Lewy Body Dementia is a, or rather, without being too sort of hair splitting on what we're talking about here, I'm going to focus on dementia with Lewy bodies, which is the version of Lewy Body Dementia that begins with very early or cognitive changes that either predate or occur with the onset of any motor changes. Because you can also get a very similar syndrome if somebody has well-established Parkinson's disease moving into a Parkinson's disease dementia. But to focus on that dementia with Lewy bodies or DLB for short, there's four core criteria from the latest diagnostic criteria and those are Parkinsonism, at least one cardinal feature of Parkinsonism, and that can be bradykinesia or a reduction in the amplitude and speed of movement, well-formed visual hallucinations, a REM sleep behavior disorder, and that's a dream enactment that occurs during REM sleep when your brain is supposed to paralyze your body so that you can't move. In this case, because of where some of the pathology occurs in DLB, it doesn't do that and so people will flail, kick, run, scream, hit in their sleep. And then finally, fluctuating cognition. There's a whole host of supportive factors in Lewy body, dementia with Lewy bodies. that range from autonomic disturbances to sensitivity to certain medications to really early and prominent neuropsychiatric or behavioral symptoms as well. Fluctuating cognition I think is the one that's hardest for people to understand and kind of get right. Parkinsonism we sort of all learn how to assess for on the exam. Acting out your dreams is another thing that's pretty easy to kind of get. There's nuances to each of them but in principle they're fairly straightforward. Fluctuating cognition as it's often described or fluctuating mentation, we often don't describe what's fluctuating. What is it that fluctuates? Because you know everybody has ups and downs, good days and bad days. With DLB, with Lewy Body Dementia, it's really the time course of that fluctuation and it's what is fluctuating. And so when I describe this to learners and to families, it's fluctuating attention and arousal. And you know, you brought up a really important point, which is kind of sounds like a UTI when people get delirium. And in fact, yeah, it looks awful lot. like a delirium that sometimes is shorter lived or unexplained but the thing that is fluctuating is the degree of arousal and wakefulness that someone has or their attention and so the questions that we ask about this are are people taking a whole bunch of naps? Are they sleepy all the time? Do they seem to be staring off and unable to get their attention very easily? are they incoherent and not really making any sense? And one question that I find helpful in clinic is folks with Alzheimer's disease or more typical forms of dementia, families will often say, yeah, they have good days and bad days. With dementia with Lewy bodies, it's very, the question is often, do they have good minutes and hours and bad minutes and hours? And so that fluctuation is on a much finer time scale. And we know that if you do this objectively, we can measure that fluctuation and attention and processing speed on every single time scale, on the millisecond time scale, minute, hour, week. That fluctuation is occurring in all of those, but it's the more, you know, within a day that seems to be more prominent.

[Dr. Mia]: 16:20

Yeah, that's a really good description of what is fluctuating because I remember one of the patient's families told me that one of the first symptoms they had was they went to get food at a restaurant and this is a woman who is in her late 50s. So by all means, she should know how to order food at a restaurant. Her husband went in front of her and ordered and then when it was time for her to order, she kind of just... zoned out. She couldn't talk at all. She looked like she was staring off into space and it was very strange and then a couple minutes later maybe she came about. Maybe there was a little bit of confusion but it really was difficult to explain and could be really scary for families to think what is going on and they're awake. standing so it's not like they collapse onto the floor and start shaking like in a seizure. But there are some, not to pretend to be a neurologist even further, there are some seizures that can have awake looking seizure episodes. So I think all of those nuances make it more challenging for people to reach the right diagnosis.

[Trey Bateman]: 17:41

I absolutely agree and there's the literature on DLB when Jim Galvin, who's now at Miami, did lead a survey of caregivers of people living with dementia with Lewy bodies and it was something on the order of 18 months and three clinicians to arrive at the diagnosis because it is complicated. It could be a bunch of different things. You know, I almost never get an EEG, which is a test that we often do for seizures, in the course of a dementia evaluation. But when I do, it's often because I can't quite tell, should I be worried this is a seizure, or should I be worried that this is DLB? And I see a lot of folks who come in having been diagnosed with a seizure disorder, and then in retrospect, it sort of becomes clear, no, they don't really have a seizure disorder. they have DLB.

[Dr. Mia]: 18:41

Yeah, yeah. And what are some differences in terms of DLB management that you think are distinct from the more typical Alzheimer's type of dementias?

[Trey Bateman]: 18:53

So, and again, I speak in a lot of metaphors. So, one of the metaphors that I tell trainees and families is that, you know, managing a lot of dementia is like trying to steer the Titanic. You know, there's the medicines we use help a bit, they move things a bit, but the effects are not large effects. it's like trying to steer a small sailboat. Little changes can make really, really big differences. And I think it's important to point out that that's both in the medicines we use and the medicines we stop using. So, you know, deprescribing is always the first thing that I think about when I see folks who are coming in with Lewy body dementia. And it really centers on the... the pathophysiology of Lewy body dementia, which is they have profound cholinergic deficits. And so we talk all the time about cholinergic drugs and Alzheimer's disease, and we talk about anticholinergics being really bad for people with dementia. The effect is almost magnified in dementia with Lewy bodies because that system is already doing worse. And so I will do everything I can to get these folks off of. of anticholinergics, which I do in all cases of dementia, but I think it's really, really critical with Lewy body dementia. And then with the treatment, cholinesterase inhibitors, which have modest efficacy data in Alzheimer's disease, I anecdotally and with smaller studies, because DLB is harder to study, I think the effect size is much, much larger. in DLB because of that fundamental failure of that system in the brain. And so cholinesterase inhibitors are really the mainstay and the first thing, things like donepezil or Rivastigmine, getting folks on that drug can be really helpful, not just for their cognition but because of where those fluctuations come from, partially from cholinergic pathology, it can help stabilize the degree of fluctuations and it can even help treat the hallucinations which seems to correlate to some degree with the burden of cholinergic deficit. So it's not so much a difference in treatment from say Alzheimer's disease because I often offer an empiric trial of that but I'm a little bit more hopeful that it's going to make a big difference early on. The other differences in DLB are that the symptoms that we're trying to manage can be kind of all over the place. So you have, on one hand, you have sleep problems. And so people have this dream enactment behavior. And in some people that can be so bad that they are falling out of bed, injuring themselves, hurting their bed partner. We try to treat that with melatonin first, but I have some patients who end up on clonazepam, which is sort of a, it's a bad drug. We don't like to use that in people with dementia. And I don't either, but in some cases, it's sort of, you've got to know what those rules are to know when you should not follow them. And so I have some patients who are on clonazepam for that REM behavior disorder because it's a huge impact on their quality of life. Similarly, I rarely use stimulant medications. Again, they're kind of bad medicines for older adults. But some of my patients that have such profound, excessive daytime sleepiness, fluctuations and arousal, the thing that's providing them the most impairment in their quality of life are those fluctuations. And so I may end up using stimulants in that case like methylphenidate or like provigil to try to help improve and palliate that symptom. Those are probably some of the biggest management differences in the medicines that I focus on. The other one that is really important is because there's often at some point agitation, behavior as the disease progresses and maybe the need for an antipsychotic. It's really really important to know whether you think somebody has dementia with Lewy bodies because many of those are as dangerous as they can be in dementia just in general. They can be really really severely damaging in patients with Lewy body dementia. The only ones that are really safe are Seroquel or Quetiapine and Clorazoril, or sorry, Clorazoril, but that one's really hard to give. And often it's only psychiatrists who are willing to use it because of the monitoring associated. But other antipsychotics can make people incredibly sleepy, more impaired, more stiff, and it's not uncommon to see people come into the hospital. in the ER they may receive, you know, Haldol. And as bad as Haldol can be, you know, all comers with dementia, it can lead to a month-long hospitalization in folks with Lewy body dementia because they're so sensitive to those meds.

[Dr. Mia]: 25:03

Absolutely and I think this is where you are an honorary geriatrician in

[Trey Bateman]: 25:07

Hehehehe

[Dr. Mia]: 25:07

talking about the medications we're stopping, the medications we should be careful about, and just knowing that anticholinergic medications are bad for memory and yet so common and a lot of times available over the counter so we don't always know if people are using it. Basically, I tell people anything that has PM in it basically is anticholinergic, meaning it's similar to Benadryl, makes you drowsy, might make you go to sleep, but can be particularly impairing or causing symptoms of memory change in older adults and even more so in people with Lewy body. And now transitioning a little bit to... talking about your research, I know you're very much interested in picking up subtle changes in terms of behavior and this concept of mild behavioral impairment. Can you tell us what that is and why you're studying it?

[Trey Bateman]: 26:08

Yeah, so mild behavioral impairment is, so mild cognitive impairment has been around for two decades or so now and it was the recognition of cognitive changes in a pre-dementia state. We recognize that neuropsychiatric or psychological and behavioral symptoms of dementia are things that occur and they're a part of the disease. And so... mild behavioral impairment is again moving that into the prodromal stage before there is a clear diagnosis of dementia. And it refers to the new onset of persistent psychiatric symptoms in later life. So by definition now these are symptoms that emerge after 50 years of age. and are persistent, meaning that they are present at least off and on for at least six months. And so this is, you know, this is the person who may have developed depression in their 50s and 60s. This is the person who may have developed, you know, even more rare, but perhaps more predictive of future cognitive disorders would be. the folks who develop later life psychosis, hallucinations and delusions. And it's not uncommon to see these people come into clinic and you do the testing and there is no cognitive disorder but there may be these emergence of late life psychiatric disease. And while it's entirely possible to develop late life psychiatric disease that is not indicative of a neurodegenerative illness. certainly wouldn't want anybody hearing me talk about this to think that everybody who gets depression for the first time in their 50s and 60s are going to develop a cognitive disorder. That's certainly not true. But there is an increased risk for developing a cognitive disorder when those late life psychiatric symptoms emerge later in life. And the reason that I'm interested in this is because it's a, we're always focused on identifying earlier and earlier time points in the course of these illnesses in the research setting. And so on one hand, I'm interested in doing that because it helps us find folks that we wanna focus on in interventional trials and the like. but I'm also very interested in being able to identify and differentiate people who've developed late life psychiatric symptoms who are going to go on to develop a cognitive disorder and those who don't. And so toward that end, we've been looking at autonomic changes in the way that people's heart rates respond during stressful circumstances. to determine is there a difference? Is there a signature or biomarker there that may help us understand folks that are at higher risk and folks that are at less risk of developing further cognitive symptoms when they have those behavioral symptoms first.

[Dr. Mia]: 29:50

Yeah, that's a fascinating joint. That's a fascinating overlap between kind of psychiatry and neurology. How much of it, how do we know what is, you know, depression, anxiety and irritability that comes very common to all of us, regardless of age and how much of it is truly a sign of something degenerative. Not. not going to get better with treatment over time. So thank you so much, Trey. Anything else that you want to add to our conversation today?

[Trey Bateman]: 30:27

Gosh, I can't think of anything else, but it was really a lot of fun to talk to you today. I really appreciate you asking me on.

[Dr. Mia]: 30:35

Yeah, thank you. And for those who are listening, I think this is a fascination that I've had in terms of memory. And this is a series of episodes. Last episode was about frontal temporal dementia. This one's about Lewy body dementia. And then I have some earlier episodes in season one talking about the two different types of Alzheimer's disease and why so many common Alzheimer drugs fail. So Thank you for listening and see you next time.