Webinar: Dementia with Lewy Bodies
Dementia Australia webinars are free videos where subject experts discuss topics relating to dementia for a general audience.
Dementia with Lewy bodies is often unrecognised or misdiagnosed, leading to problems with prognosis and management.
In this webinar, Professor Simon Lewis highlights some of the challenges posed by the variety of ways in which Dementia with Lewy bodies can present, as well as common symptoms and their management.
You’ll learn:
- the variety of ways dementia with Lewy bodies can present
- why dementia with Lewy bodies is commonly mis-diagnosed
- common symptoms and their management.
Transcript
[Beginning of recorded material]
[Title card: Dementia Australia]
[Title card: Dementia with Lewy Bodies]
Simon: Hello, I'm Professor Simon Lewis at the University of Sydney Brain and Mind Centre, and I'm delighted to have the opportunity to tell you all about dementia with lewy bodies. I'm going to start with an acknowledgement of country, and I'd like to recognise the traditional owners of the land on which we meet today, and also pay my respects to Elders, past and present, and to our shared futures. With this presentation, what I'd like to do is to start off with some cases just to give you a little idea about where we come out from making a diagnosis of dementia with Lewy bodies, and then really talk about some of the management aspects. Impossible to cover everything in such a short period of time, but hopefully, by the end of this session, you'll be able to get a lot better feel for this condition. And you'll also see on this slide my website, where there's a bunch of free resources where people can go and watch videos and get some more information.
So, let's go to clinic. Let's start in the geriatrics clinic where a patient has gone in. It's a lady who's 70 years old and her daughter is concerned about her, and this is often the case, that it's not actually the patient, but a family member who's raised the concern. And she said, "Look, over the last six months, mum is just getting a bit more forgetful. We're having to remind her about conversation, remind her about appointments. And she's also had this funny thing where she sort of zones out. She goes blank. It's almost as though she's in another world." It's often the case with these patients that they'll sometimes getting taken to the emergency department for these episodes where it looks like they might be having a little seizure, or some kind of mini-stroke, and that's what people often think they've had and they go along, but this is what she was concerned about, “mum's just sort of zoning out on me and forgetful”, and occasionally, she sees her mum having a bit of a shake, usually, when she's just drinking a cup of tea or holding a newspaper. So that's our patient in geriatrics.
Now, next door, we've got the neurology clinic, but there's another 70-year-old patient who's come along with his daughter, and she's concerned that for the last six months, she's noticed some physical changes with him. He's walking more slowly, and sometimes, it looks like he's shuffling with a bit of a stoop, but also concerningly, when we take the history, it finds out that he actually started to see people lurking around in the garden and they're not really there. So, we're getting this report of, if you like, visual hallucinations and some of this change of physical symptoms.
And then, if we go next door again and we see our colleagues from sleep medicine, they're in there with another patient. She's also 70 years old, and she's been brought in to see the sleep doctor because she fell out of bed and broke her wrist. She actually punched the wall while she was sleeping and that's why she's managed to break her wrist. And her husband, when you take the history from him, says, "Look, actually, she's been doing funny things in her sleep for about 10 years, and she'll scream out loud, she'll sometimes wave her arms, and this is the most violent episode she's had where she'd actually managed to punch the wall. And she's had one episode of falling out of bed."
In addition, when you probe the history, we find that she can actually no longer send a text from a mobile phone. She could do that a couple of years ago, but in the last six to 12 months, she's just getting bamboozled by the technology. So here, we see three different clinics, and in actual fact, there are lots of other places that patients can present to. And so, some patients will present through having had a routine operation with a delirium. Some patients will present to the old age psychiatry team, perhaps with some late onset psychotic features like delusions or hallucinations, but these are just three examples, and interestingly, they're all examples that fit nicely with what we see in dementia with Lewy bodies, or DLB.
So, what we're talking about here is, essentially, a condition where there is a progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational function. So this is, if you like, a cognitive decline that's impacting on activities of daily living. And when we look at the memory testing profile, we'll often find deficits on attention to the ability to attend to information, and change attention between various things. Executive functions, so working things out is your perceptual ability. So often, complaints about driving, so the car veering off towards the middle of the motorway, or parking and scraping the car; they weren't problems before. And they're often the more prominent issues, rather than pure memory problems, but we do see memory problems in DLB.
And important to note that these are things that seem to come at a pace, so somebody is obviously getting worse over 12 months, and that can be combined with some of those other features that we saw in the clinic across those three different patients: physical symptoms, sleep symptoms, and a range of other things. So, the core features that we're looking for to support the diagnosis in addition to this cognitive decline that's, if you like, rapidly coming over 12 months are fluctuating cognition. And so, this is with pronounced variations in attention, alertness, that patient that we were talking about who zones out, it might be just for a fraction of a second, it might be for a few minutes, and sometimes, as I say, severe enough that they get taken to the emergency department. Often, they will have normal brain scan, brainwave studies, and be told, "Oh, you had a mini stroke. We don't know what happened." And often, by the time they're seen by the doctors, they're back to normal.
The other features include recurrent visual hallucinations, typically well-formed and detailed. So often, it's going to be people hanging around. Children are common. Animals, so cats, rats, cockroaches, mice, ants, these things that are the stories that we often hear with people having these recurrent visual hallucinations. Spontaneous features of parkinsonism. Now, parkinsonism reflects those features that we see in someone with Parkinson's disease, but you don't have to have the same features, or all of the features necessary that you see in Parkinson's. So, any kind of tremor that's come on that's new would be regarded as suspicious. Any change in balance or gait disturbance would be suspicious. These are features that don't have to fulfil the diagnostic criteria of Parkinson's disease, but have to be a change. So, if someone's always had a tremor since they were in their twenties, you might disregard that, but if they've only developed a tremor in the last year or so, then you might take more attention.
And finally, acting out the dreams. So, this is rapid eye movement sleep behaviour disorder. So people, when we're asleep, we transition through different stages of sleep, and one of those stages is dream sleep when our brain is very active. But there's a mechanism in the brain that basically paralyses the body so it goes limp, and shacked out those dreams. And in DLB, as well as with Parkinson's, and some rarer conditions, we see this mechanism breaking down in the brain, and often, even before the memory changes occur or physical symptoms occur, patients are reporting acting out their dreams.
And if you haven't seen it, or perhaps you are familiar with it, it looks a bit like this in the video I've got next. This comes from the Mayo Clinic in the States, which is a video of patients who, through this whole recording is asleep, but they happen to be in dream sleep. And what you can see is that their body is not paralysed. they're actually acting out their dreams and, of course, a lot of dreams are quite violent in their content. People are often playing sport, or avoiding being hit, or in a fight themselves. And so, these things can be quite dangerous and a number of patients will actually injure themselves or injure their bed partner.
This is a very key feature because it's a very distinctive feature as opposed to most of the other illnesses with the dementia. And this is a study done of over 170 patients who, during life, acted out their dreams and then after they passed away, their brain was examined. And this is a busy next slide, but really what it says is that the vast majority of people that act out their dreams, over 95% of them who do this dream behaviour, and you can't find another reason for it, will have something like DLB or Parkinson's, these related conditions where that pathology affects the part of the brain that stops people from acting out their dreams. So, if you hear someone who has a dementia, and you hear that they're acting out their dreams, either it's recently started or it's been going on for five, 10 years or so, the likelihood is that you're looking at someone with DLB. And that's really important because we just, as you see from this slide, really don't see this story in patients with Alzheimer's disease commonly.
So, what else about a dementia with Lewy bodies? So probable DLB can be diagnosed if we've got two or more of those core features. As I say, those fluctuations, the hallucinations, the physical parkinsonism, and the acting out the dreams. So, if you've got two or more of those, plus this cognitive decline in the first 12 months, we can make the diagnosis of probable DLB.If you've got less than that only, say, one core clinical feature, perhaps with something supportive like a sleep study that shows you're acting out your dreams, then actually, we can make the diagnosis of possible dementia with Lewy bodies. And people are looking harder at trying to diagnose DLB before it's, if you like, become established, what we call prodromal DLB. And there are a number of researchers who are currently looking to see are there patients that we can predict who will gone to have DLB, like those people who act out their dreams, who might be getting early cognitive changes.
Possible DLB, as I say, you need to have just one feature and some biomarkers like that sleep study, and you should never diagnose probable DLB just using those things like a test, like a brain scanner, and a sleep study. Typically, it affects people as they get older, so over the age of 60. It's very rare, in fact, I don't remember seeing a case of DLB under the age of 60. And if it is, there's often a gene that's in there, and the gene cases are rare. It's said to account for about one in five patients with late onset dementia, so second only to Alzheimer's dementia. More men than women, and we don't know the reason for that, whether there's something protective about female hormones and the process underlying the pathology of DLB is something we're exploring.
And what does it look like? Well, clinically, it looks like a cross between Alzheimer's, Parkinson's with patients who develop memory problems, and physical problems. And indeed, in advanced Parkinson's, we see all of the features that we see in DLB. We just see them after the disease has being there for maybe 5, 10, 15 years, depending on how old the patient is when they get their diagnosis. So, we're often seeing this, if you like, mixture between cognitive and physical symptoms mixed in with those other features affecting sleep, for example, and hallucinations.
It also is the same under the microscope. So, we actually see a bit of a mixture of the pathologies of Alzheimer's and DLB, a more aggressive disease, and I'll talk a bit more about that pathology in a second, but a more aggressive disease than we see in Alzheimer's. The prognosis, they say, normally, we talk about a survival of two to six years, but remember, many of these patients are diagnosed in their eighties, so in actual fact, they've already, if you like, exceeded what one might have predicted for their expected life duration. And as I mentioned, much more impactful than Alzheimer's dementia, more costs, faster cognitive decline, more likelihood of going to a nursing home, caregiver burden, and worse survival rates.
And it is diagnostically challenging. Even when we see the brain underneath the microscope, there's often a mixture of pathologies. So, at one end, you might see something like Parkinson's purely, and then Parkinson's dementia as the disease progresses. DLB with this very clear pathology that looks like Parkinson's with tangles of a protein called alpha-synuclein. So down the microscope, you find these alpha-synuclein clumps, what we call Lewy bodies, sitting inside the dying brain cells. But even in people with pure dementia with Lewy bodies, you'll see other pathologies, so a mixture of Alzheimer pathology in there with some of the proteins that we see in Alzheimer's also mixed into the brain, so the amyloid protein in particular.
Outside of the cells, we'll most often see a lot of that in patients who got to post-mortem who've got DLB. You see less in the way of full-blown Alzheimer's dementia change with all of this other tau protein inside the neurons with brain cells. But interestingly, in patients with Alzheimer's dementia, clear Alzheimer's, when you often find a few little evidence traces of Lewy bodies. So, there's this real spectrum that makes it hard for us clinically, and also hard for how we come up with good treatments.
How do we tune into making the diagnosis? Well, I mentioned earlier that cognitive decline is the essential part about making the diagnosis and it's usually rapid. Within the first 12 months of noticing problems, there is this obvious change. Memory being just one aspect, attention to being able to focus on a task, switch tasks, working memory, trying to work things out in advance, and often, visuospatial problems like I mentioned with driving, for example. Languages, services, less common in dementia with Lewy bodies, but still possible. So not being able to find the right word in a conversation would be a softer example of what we see. And the pattern of cognitive decline. Alzheimer's disease, we typically see people who we had a major memory problem and we call this amnestic. It's little bit like the goldfish in the bowl where, in fact, we can't lay down a new memory. You might remember things from the very distant past, but if we gave you, say, a list of items to recall, and then asked you again for those items a few minutes later, the patients will really struggle with that laying down new information and new memories.
DLB is less so, but not exclusively less so on memory, but more on attention, working memory, visuospatial, so these non-amnestic features, but we certainly do see memory problems, especially as the disease progresses. And there are patients out there who, early on, present with memory problems as their florid feature.
There are also a number of nonphysical symptoms like these cognitive fluctuations where patients are zoning out and will go unresponsive that we've touched on earlier. Dream enactment, which I've shown you the video of people acting out their dreams, punching, kicking. We do see dream enactment in other conditions, so severe sleep apnea can do this, people who drink too much alcohol, perhaps late at night, certain medications, some of the antidepressants can do this, and some illicit substances can do this. And we're not talking about people who go sleepwalking. So, people who take a walk in their sleep, that's a very different thing. We're talking about people who basically act out their dreams, and they generally don't get much further than the bed they're lying in because they'll fall over and hurt themselves.
Visual hallucinations, as I mentioned, typically, it's people. Children are common, animals are common, and often not threatening. Patients will say, "Look, I see them. I try to talk to them, and they disappear. I reach for them, and they disappear." And as the disease progresses, these people can get more threatening and that's when things get difficult because we see accompanying the hallucinations more in the way of delusions. So, delusion is where you believe something to be true isn't true. Hallucinations is seeing something that isn't real. A delusion, typically, is restricted in dementia with Lewy bodies. We often see, typically, things like somebody is breaking into my house, somebody is stealing my stuff, my partner is having an affair, someone is trying to poison me with these medications. It generally is that sort of repertoire. Sometimes, we'll say, "Look, I recognise my husband, but he's not the same person. He's a different... he's impersonating my husband," a very specific type of delusion, which you can see across the dementias, but it's something that we tune into in DLB.
And then these physical symptoms, so non-motor but now motor, as we call it in neurology, parkinsonism. So, things like a shuffling gait, slowness, stooped posture, tremor. That doesn't have to be when the patient's just sitting at rest, but can be when the patient is using their hands to do something like drink or eat. What about investigating dementia with Lewy bodies? Well, obviously, memory testing plays its role, and there is this argument about bedside testing where you go to see the doctor, or a nurse, or somebody on the ward, You can sit there for half an hour or 20 minutes depending on how good someone is and say, "Hey, can you come up with a list of this? Can you draw that for me? Can you take this number away from that number?" And that is usually very useful, but also, you can go into much more detailed neuropsychometry, two, three hours of testing into very detailed domains of cognition across those things that we've talked about like attention and working memory, language, visuospatial, and memory.
Investigating with blood tests. Well, there's a typical dementia screen that a GP will do. Does the patient have anything else that might explain the cause of dementia? Traditionally, we've thought about diseases like syphilis back a couple hundred years ago. We still do tests for that, but also, vitamin deficiencies, other things that might give us a clue as to why someone's memories potentially go down and potentially reversible. Other tests, brain scanning, generally making sure that you haven't got something else. You don't want to find somebody has a brain tumour that might be treatable, for example. You can also look at the pattern of shrinkage of the brain, and typically, in early cases, we don't see any that's very helpful, very diagnostic. But in Alzheimer's, we see certain parts of the brain involved in memory seem to shrink more than we would see in patients with DLB, for example, but not diagnostic. And I mentioned the need for PSG, which is a sleep study, a polysomnogram, and really, what we're talking about here is something that has to happen, a specialist centre where they all put on special electrodes to record the muscle activity in certain areas like the chin, and the arms, and the legs. This is the sort of thing that doesn't happen with a home sleep study, for example. Really important as well to get a video of that night's study because, of course, if people are acting out their dreams, you really want to see what their behaviours are doing.
In terms of treating DLB, I'll cut to the chase, we don't have a cure, and we don't have anything that slows the course of the disease, but we'll mention that a bit later at the end, perhaps. Treating physical symptoms can happen. We talked about parkinsonism, so we tend to use other Parkinson drugs, because a number of patients will improve with physical symptoms in the response to a Parkinson's treatment. But in addition, there's all the things that's mandatory, so rehabilitation – physiotherapy, exercise therapy, working on core strength and balance, as well as speech therapy, and in addition, things like levodopa, the medication we use for Parkinson's can also benefit some of these symptoms and reduce the risk of falls.
Generally, we give lower doses than we would in Parkinson's disease because we don't want to get side effects, and we go slowly when we're escalating the doses. And if we do see things getting worse like sleepiness or blood pressure, then obviously, we have to pull back on those treatments. Treating memory is more difficult, so we try to address aggravating problems, so complicating medications, poor sleep, sleep apnea, mood disturbances, all worth treating. And non-pharmacological approaches, so counselling, changing the home environment to make things simpler so you know where the toilet is, you know where the crockery is put away. Cognitive training, whether it's online and in person, can be helpful. And then there are medications, the drugs again, some from the wood of Alzheimer's, which we do see giving modest improvements for things like memory, but also, for hallucinations. And indeed, in treating psychiatry, we can, as I say, focus on these symptoms. We try and avoid the traditional antipsychotic medications that are used for schizophrenia, and use the more atypical drugs that although they have risks associated with increased cardiovascular death, that's a very low risk compared to patients who become agitated and aggressive, and want to remain in their own home, we have to balance that risk out.
And as I mentioned, just cholinesterase inhibitors from memory can also improve the reduction of hallucinations. And hopefully, there are new drugs that have been approved elsewhere in the world that might come through into practise in Australia. Treating mood is very important and counselling can be useful here, as well as the medications that we typically use in treating mood disturbance. Autonomic, so fainting, so this is the autonomic nervous system, things you don't think about doing, so fainting, the blood pressure, constipation, bladder disturbances, drooling, pain, these are all features that we should look for and aggressively treat, because we do have some treatments that can help our patients with these symptoms.
The sleep-wake problem, so sleepiness during the day or somnolence, and we want to rule out things like sleep apnea, make sure they're not on medications that make your patient drowsy, and maybe consider using drugs to wake people up. Now, the dream behaviour, we need to make the bed environment safer, so no glass or other things that are breakable by the side of the bed, perhaps even some foam by the side of the bed or a lower bed if people fall out of bed regularly. Medications for that dream acting behaviour are tricky. The drug trials don't really support anything at this time. Novel therapies, there are things being developed for psychosis, for memory, and disease modifying therapies. We're hoping that trials will start to come along and certainly, we have great interest in trialling these approaches. But we need more funding and more support.
In summary, DLB is common. It's a clinical diagnosis at the moment where you can't just have a blood test or a brain scan. There's a significant overlap with the number of the other conditions that can get confused with DLB in the clinic. There are a limited number of investigations, on top of cognitive testing that we should think about doing, and the management strategies at the moment are largely supported. With that, I'm going to pause, and put this slide up to remind me to thank my team of researchers who are doing a great job, and they do all the hard work, which allows me to come along and give presentations like this today.
[Title card: Together we can reshape the impact of dementia]
[Title card: Dementia Australia. 1800 100 500. Dementia.org.au]
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