3 Dementia Lewy Bodies | Atypical Parkinsonism (DLB, PSP, MSA, CBS/CBD) Symposium

good morning what a great looking crowd alert but you didn't have to get up as early as I did they made us be here at 7:00 and we don't all live next door I'm Karen Duncan I'm going to be the moderator of this session and this is the dementia with Lewy bodies session I'm told to say a word about my own connection to the topic I joined Robin riddles a typical parkinsonism support group shortly after my husband was diagnosed with Lewy body in 2004 and he died in 2006 and his diagnosis was confirmed through brain donation as you know the original support group has just recently incorporated and become the brain support network so that Robin can raise money to support the process of getting the getting brain donations from throughout the country and I'm very pleased to tell you that I'm a member of the founding board of directors of the brain support network I think it's going to be a lot of fun our speaker is going to speak until 11:35 and then we will have the 25 minutes of questions which will take us right up to noon lunch now I would ask that you get your questions in as soon as you think of them because I have to sit up here and sort through them while trying to listen to him at the same time so just raise your hand you don't have to get up just raise your hand and the gentleman in the back of the room will come and get the questions and bring them up to me and you can do this throughout the lecture now I'm going to introduce our speaker who's dr. Jeffrey Kirchner who's assistant professor of neurology at the Stanford School of Medicine Stanford University School of Medicine his clinical focuses focus includes Alzheimer's disease dementia behavioral neurology neurodegenerative disease neuro psychology neurology and mild cognitive impairment so you're going to be hard-put to think of a question he doesn't have an answer to his research interests as a behavioral neurologist are in caring for patient with Alzheimer's and other age-related neurodegenerative illnesses his study he studies the use of MRI and other advanced neuroimaging technologies with the intent of creating new strategies for early diagnosis this is a special interest to me because my husband had symptoms for at least eleven years before he was ever diagnosed and who knew what was going on dr. Kirchner received his MD and PhD degrees from Washington University he did his residency at UCSF and his board certified in neurology and he's received many professional awards and has published extensively so this morning dr. Kirchner is going to bring us up to date on the treatment of and research on dementia with Lewy bodies wonderful thank you very much can everybody hear me okay okay good if for some reason this microphone gives out just let me know so I want to talk to you about the most common disease that nobody's heard of so many doctors that you go to primary care doctors have never heard of dementia with Lewy bodies but it is at least the third most common cause of age-related cognitive decline in people over the age of 65 it's an incredibly common illness often times it's not diagnosed until autopsy that's something that I and many other researchers want to change because I do think that there is a lot of value and being able to get the right diagnosis during life so that's part of what I'm going to talk with you about today but I wanted to start by talking about if I can get this to work I might have to push a button there we go all right it's not quite a wake yet on the Saturday morning so let me talk to you about what dementia with Lewy bodies is in the first place and I want to emphasize that these criteria that you've heard about and you've seen lists of and that I'm going to take you through in detail these criteria are not designed to help a doctor in the clinic so much as they are designed to help researchers assemble a group of patients who are as much alike to each other as possible in order to do research studies on a more or less homogeneous patient population so I want you to understand these criteria and any other criteria that people tell you about other illnesses with a little bit of a grain of salt because it's not the case that if you don't meet these that you absolutely do not have dementia with Lewy bodies okay so with that grain of salt let's go through these and understand what the core features of this illness actually are so the first one the first thing that you have to have in order to be diagnosed with dementia with Lewy bodies is dementia so let's take just a second to talk about what is dementia because that's a word that is not very well defined in the popular media the newspapers get it wrong people out there in the community get this word wrong I think sometimes and I can't tell you how often in my clinic my patients asked me is this dementia or is it Alzheimer's disease and so that reflects I think in a misunderstanding of what this word is so to me the word dementia means simply this a gradual unexplained loss of cognitive skills that is severe enough to cause an inability to function in everyday life okay it's not a disease dementia is not a disease it's a state of being it dementia just as a way of describing that somebody used to be II this high and intelligence and they've come down there's been a decline and the word is meant to communicate the level of severity of that decline in other words it's been it's become so bad that that individual was now has to for instance give up a job or now can't be trusted to manage the bank account by themselves something they've had to give up that they used to be able to do some aspect of their ability to function independently has had to be given up so that is what dimension means there are lots so cognitive skills that term is very broadly defined it can include executive functioning which is a term that's meant to describe problem-solving skills the ability to think on your feet the ability to pay attention and stay focused on something and avoid distraction judgment those kinds of skills are subsumed under this term executive skill executive function visual spatial function is another kind of cognitive skill this has to do with navigating being able to make sense out of a three-dimensional world around you being able to judge distances and the motion of objects being able to open the refrigerator door and the ketchup is right in front of you but there's also a lot of other stuff and being able to find that bottle of ketchup in the middle of everything else that's that's a complex visual spatial skill that your brain has to be able to do memory is another cognitive domain being able to remember something that you and your husband talked about five minutes ago being able to encode new information to write new information to your brains hard disk drive that's what memory is language being able to think of words can you hand me that um that oh you know that thing over there that sort of stuff and language has to do with other skills to being able to express words correctly so these are just examples of different aspects of cognitive skills that might contribute to somebody's diagnosis of dementia and I put stars next to executive function and visual spatial function because these are the two areas that are often not always but often most affected in dementia with Lewy bodies okay so we're talking about the diagnostic criteria you have to have dementia which is what I just talked about and then you are supposed to have two out of three of the following core features so let's talk about these these core features are important because many patients with dementia with Lewy bodies have some aspect of two or three of these the first one is fluctuating attention and concentration now all of us have good days and bad days if you put me on a flight to Korea and you know I'm supposed to wake up and give a talk the next day I'm not going to be mentally as with it as I usually am I'm going to be sleep-deprived and grouchy and all that stuff so we all have some fluctuations in our ability to to be intelligent but for a patient with dementia with Lewy bodies this is profound typically patients will be anywhere from being absolutely confused no idea when it is where it is what's going on right now to being relatively normal and and fluctuating kind of unpredictably between those two ends of the poles I remember one patient that I had in clinic who was generally fine he and his wife were planning a trip to New York to go visit their daughter and on the airplane he became he went berserk he was absolutely confused didn't know where he was and and really raised a stink on that airplane flight and that's just an example of the kind of the amplitude of confusion that a patient might experience so the next one is recurrent well-formed visual hallucinations so let's talk about what this means so first of all what is a hallucination so a hallucination is a is a false sensory experience thinking that you see something and in dementia with Lewy bodies it almost always has to do with vision examples are I'm sitting here in the sofa my wife sitting next to me I know that she got up and walked to the kitchen but I'm looking and I still see her sitting in the sofa next to me even though I know she's not there I might even reach out and try to touch her but I see it so it's it's a to the patient it is absolutely real the patient might have insight they might know that this can't possibly be real I know that my mind's playing tricks on me but and yet it looks like my wife is literally sitting there or there are people sitting around the dining table you know there's a whole family sitting here around the dining table we're having a meeting and the wife's trying to explain to her husband that that you know we're having a meeting right now and the husband is befuddled there's nobody here but to the but to the patient this is a real experience they're seeing these often people sometimes objects but it's often people classically a patient with dementia with Lewy bodies at the beginning of the illness these can be images off in the corner of the vision faces on the wall is a classic thing that people describe often times seeing things through the window you look out through the window and there's a little family out there playing on the on the on the on the front lawn sometimes there are small animals or magical creatures unicorns or things like that so there's a wide variety of things that people might see that's what a hallucination is it's a little bit different from an illusion which a patient with dementia with Lewy bodies might experience to an illusion is when you look on the floor and you see a little kitty cat over there and you you walk over and you reach out to pet it and it's actually just a shoebox it looked like a kitty cat but it was it's just a shoebox that that's an illusion where you actually see something there's there's something actually there but your mind is giving you wrong information about what that thing is and in dementia with Lewy bodies there's often a misperception that's something that's not animated something like a stone or a shoebox or an inanimate object is actually a living creature that's a common phenomenon in dementia with Lewy bodies there's this other term delusion delusion is different from these visual experiences a delusion happens when a patient true strongly believes something to be the case that's not really true my husband's having an affair I'm quite sure I've I'm just I have no idea why I think that I'm just absolutely convinced of it and nobody's going to talk me out of this so delusions are these false beliefs that a patient might develop and so all of these might occur in dementia with Lewy bodies good so two out of three of the following fluctuating attention and concentration recurrent visual hallucinations and spontaneous parkinsonian signs so what's a parkinsonian sign so these are all the symptoms of Parkinson's disease some combination of tremor stiffness balance problems and slowness so those are the four cardinal features of Parkinson's disease as we're going to talk about dementia with Lewy bodies is very closely related and in fact is on a spectrum with Parkinson's disease and so all of the exact same symptoms that you can see in Parkinson's disease characteristically happened at some point during the course of the illness and dementia with Lewy bodies okay so there are some other features some other aspects of dementia with Lewy bodies that are that are common enough that they show up in these diagnostic criteria those include what are called rapid eye movement sleep behavior disorders now what the heck is that so almost everybody with dementia with Lewy bodies has experienced this at some point well the patient may not experience it but their husband or wife knows exactly what this is in the middle of a dream you're dreaming about somebody breaking into your house and most of us our brains are designed to paralyze the body so that during dreams during a dream your brain can't tell the difference between the fact that this is a dream versus reality the brain is actually awake and you can record the the fact that the brain is awake within with an EEG you look at the electrical activity in the brain the brain is awake it's truly experiencing this burglar coming into the house and your brain is activating your legs to move and your arms to move your brain is doing all this stuff but our brains have developed so that when you're dreaming your body becomes paralyzed so that you don't actually carry out the things that your dream about but a patient with Parkinson's disease and dementia with Lewy bodies that paralysis mechanism doesn't work very well so that some of those commands to move your arms and move your legs actually make it down to your arms and legs and you start fighting off the burglar in your sleep and unfortunately that's usually your wife or your husband that you start punching and kicking and and that kind of stuff so that's that's an example of of what's meant by a rapid eye movement sleep behavior disorder a failure of that paralysis mechanism to work in the middle of sleep and some people think that this actually precedes almost all of the other symptoms of Parkinson's disease by something like a decade on average so that REM sleep behaviors are actually fairly common out there in the population and many scientists believe that patients who have that problem will eventually if they live long enough go on to get Parkinson's disease or dementia with Lewy bodies that it's part of a disease spectrum sometimes the disease is slow enough so that you end up dying of something else before you ever get the symptoms of Parkinson's or dlb another very common feature of dementia with Lewy bodies is severe neuroleptics sensitivity what does that mean that means that when a doctor gives you an anti-psychotic medicine to try to make those hallucinations go away you don't do very well with that medicine you become severely parkinsonian all that stiffness and tremor and rigidity becomes severely bad it's a common side effect of those medicines but that that side effect is amplified tenfold and a patient with dementia with Lewy bodies so that they often can't tolerate those anti psychotic or neuroleptic medicines very well and then a final clinical feature has to do with a brain imaging technology where you can dopamine is a neurotransmitter it's a chemical in the brain that becomes messed up in both Parkinson's disease and dementia with Lewy bodies and you can visualize some of the machinery that has to do with regulating dopamine in the brain and there are differences between normal people and and people with the illness in terms of how this machinery looks so this is just an example of a brain scan for these so called dopamine transporters now this is a normal patient and you see all these hotspots and what a part of the brain called the basal ganglia and also down here in a part of the brain called the substantia so in this particular patient there are nice bright hotspots where these dopamine transporters are located and this is a healthy brain and a patient with dementia with Lewy bodies you might see a deficit a reduction in in that hotspot signal there are a lot of other symptoms that a patient with dementia with Lewy bodies might suffer depression and anxiety are common and I think that anxiety a worsened anxiety and somebody who may have had an anxiety problem earlier in life or a brand new symptom of anxiety and somebody who is always even-tempered before I think that that can often precede other symptoms in the disease by some years orthis stasis which is a fancy way of saying that you get dizzy when you stand up too fast that's another common symptom in dementia with Lewy bodies I told you all about visual hallucinations but people can have non visual hallucinations to hearing sounds that aren't really true feeling things like bugs crawling on my skin to the point that I'm slapping my skin and I'm putting on you know really poisonous lotions to try to kill that I mean really it being absolutely convinced that there are things crawling in your skin delusions I've already told you about loss of consciousness passing out a lot this can be a symptom sometimes in a few patients with dementia with Lewy bodies so here's a big question that I get a lot in my clinics so I see patients who are who are having some problem with their memory or other cognitive skills and sometimes problems with walking and the doctors who send those patients to me are asking me to figure out what is the right diagnosis and I wonder if any of you in the room have been through the experience of trying to figure out what's the right diagnosis for yourselves or for your loved ones and you know better than anybody that this is a challenging problem and there are a lot of terms that doctors will throw around is it dementia with Lewy bodies is it Parkinson's disease dementia is this actually Alzheimer's disease or maybe it's something completely different and doc you two or three different neurologists you'll get four or five different opinions this is a problem in the field it's a problem that we're trying to solve so as you heard dr. Littman speak about earlier the only way to be absolutely sure of what the illness is is to and I tell my patients this we can chop your head off cut your head open take your brain slice it up into thin slices and look at it under the microscope and then I can be absolutely sure what's the matter with you but I haven't had any volunteers for that yet but in all seriousness I think that we do learn a lot from doing autopsies have you ever heard the term senile dementia or hardening of the arteries so these terms used to be around in the 50s and 60s and 70s and 80s so many of our grandmothers and grandfathers were you know maybe diagnosed with senile dementia or hardening of the arteries when they started losing their memory as they got old well it wasn't until people really started doing autopsies on Grandma and Grandpa that they figured out that this is actually Alzheimer's disease did you know that in the early part of the 20th you know really into the 1970s or so Alzheimer's disease was believed to be enix extremely rare disease of young people because that's how it was first described and what because nobody ever did autopsies on Grandma and Grandpa but when they finally did they realized that diseases like Alzheimer's disease and dementia with Lewy bodies ended up you know being the underlying problem that that caught that led to memory decline with age so autopsy is very important for us to really understand these illnesses but that's the way to be absolutely sure of what's the matter let me focus first on this distinction between dementia with Lewy bodies and Parkinson's disease dementia as anybody in the room just show up your hand ever been confused about the difference between these or have doctors tell you that it's one or the other okay good well the doctors are confused about this too honestly and that's why you're getting such a mixture of opinions I think the truth is that these diseases exist on a spectrum with each other now there are funny rules like if you have your cognitive symptoms for at least one year before the first time anybody notices tremor or stiffness or slowness those parkinsonian symptoms then we call it dementia with Lewy bodies but if a tremor shows up within one year or before the cognitive symptoms show up then we call it dementia with Lewy bodies this is arbitrary people these are not distinct diseases and in fact a pathologist looking under the microscope would it would be hard-pressed to to tell the difference between them the pathologist wouldn't be able to take a brain of one patient who was told they met research criteria for Parkinson's disease dementia and another brain of a patient who met research criteria for dementia with Lewy bodies the pathologist wouldn't be able to tell the difference between the two brains they look the same under the microscope and in fact many pathologists argue that they can't even tell the difference between a patient who has dementia with Lewy bodies and a patient who has plain-old Parkinson's two without any of the cognitive symptoms and so what that suggests to me is that actually all these diseases are on a spectrum with each other now I'm drawing this spectrum from subcortical that is that's just a fancy way of saying parts of the brain that are in the middle as opposed to around the outside Parkinson's disease is when only those middle parts of the brain are symptomatically affected dementia with Lewy bodies is when it's really the outside of the brain the cognitive parts of the brain that are most significantly affected in Parkinson's disease dementia is somewhere in between where both parts of the brain are somehow equally affected but again these are hard to tell apart and probably these are not distinct diseases there are just different sides of the same coin okay what about Alzheimer's disease so this is something that we're actively learning more and more about a dirty secret in the field very dirty secret don't tell anybody a dirty secret is that a neuro pathologist looking at you know the brains of patients who have died with dementia if dementia with Lewy bodies is present okay virtually every single one of the BRIT of those brains will have some degree of plaques and tangles those are the features of Alzheimer's disease in other words almost every brain of a patient with dementia with Lewy bodies will have some aspect of Alzheimer's disease present in the brain - anywhere from fairly mild to being absolutely blatant like being indistinguishable from a patient who dies of you know purely Alzheimer's disease and so it's the rule rather than the exception that there tend to be mixed pathologies at autopsy patients tend to have more than one thing it's very very difficult in life to tell the difference between whether a patient's symptoms are mostly due to Alzheimer's disease or mostly due to dementia with Lewy bodies it can be hard to tell the difference now Alzheimer's disease the symptoms are a little bit different you don't tend to get so many visual hallucinations and you don't tend to get such wide fluctuations in your attention rather short-term memory is usually the main problem and occasionally in clinic I run into patients who very clearly fit into one bin or the other where short-term memory is their main or their only problem or patients who have normal memory but have flagrant visual hallucinations parkinsonian symptoms and seem clearly to have dementia with Lewy bodies but those patients are relatively uncommon in more patients who I see in clinic have a little bit of a mixture of those symptoms and that makes it hard for me when families ask me is this Alzheimer's or is this dementia with Lewy bodies I have to be honest with many many of my families and patients and tell them I'm not sure because that's the honest truth dr. Zin and if a doctor tells you if a neurologist tells you I'm a hundred percent sure I know what this is he's not telling you the truth or he's or he's falsely convincing himself that he knows something that he doesn't because we're not that good the doctor might be having a hallucination so vascular dementia so I mentioned that dementia with Lewy bodies is the third most common type of cognitive decline Alzheimer's being the first Alzheimer's disease accounts for about two-thirds of cases of cognitive decline vascular dementia is the second leading cause everybody who gets up to 65 70 or so has some ratty-looking blood vessels in the middle of their brain it's very hard to get up to that age without getting some damage some wear and tear on your blood vessels now sometimes people have an actual stroke and that's a little bit different a stroke happens when you have weakness on one side of your body or the other I mean you you know it when you have a stroke and sometimes patients can get what's called small vessel disease just ratty blood vessels in the middle of their brain that a doctor might notice on a brain MRI the doctor looks at the MRI and they see those those ratty looking blood vessels in the middle of the brain and they say well you've got white matter disease or you have small vessel disease and this is also on a spectrum in virtually every patient who I see with either dementia with Lewy bodies or Parkinson's or Alzheimer's disease has some degree of small vessel disease in their brain - and it's impossible to tell to what degree - those ratty blood vessels contribute to some of the cognitive decline and the honest truth is that for a patient who say 80 years old and has some cognitive decline and some including memory problems and visuospatial problems in some Parkinson's the honest truth is that that patient probably has a mixture of all these things okay that so unfortunately when we get to a certain age it's perfectly plausible that you can have more than one thing wrong with you and I think that that's often the case alright let's talk a little bit about treatments so getting the diagnosis right is something that it's something that you all want to know it's something that I as a scientist want to know because it gives me pleasure to get the answer right I love getting the answer right the honest truth is that it doesn't make that much difference for the patient because we don't yet have treatments that are specific for one disease or the other disease and so it doesn't actually make all that much difference in terms of what the doctor does for the patient and in terms of what you as a caregiver do for your loved one it doesn't make that much difference because the rules that I'm about to tell you apply whether or not you have the diagnosis exactly right or not for dementia with Lewy bodies the first rule above all when you go see your doctor is avoid bad drugs okay I spend more time in my clinic taking people off of drugs than I do putting them on drugs okay what do I mean by this there are some very innocent medicines that you can get over-the-counter at Walgreens anything that's labeled as PM like Tylenol PM or Advil PM anything that says PM any allergy medicine any over-the-counter sleeping medicine all of these over-the-counter medicines contain some sort of antihistamine drug in them in fact you can tell this because you can flip it over put on your reading glasses and look at the small print on the back of the box and it'll give you a long chemical name dot dot dot and antihistamine look for that because antihistamines are awful for patients with dementia with Lewy bodies they make people confused they can sometimes even put them in the hospital with such severe confusion because antihistamines block one of the key neurotransmitters in the bleh in the brain that keep you alert awake focused paying attention and these antihistamine drugs knock down that chemical a patient with dementia with Lewy bodies is hanging on to the last little tiny teaspoon of that chemical that they have in their brain just to keep their wits about them now the rest of I can take a benadryl and I'm not going to feel great I'm going to feel a little bit you know giddy or drowsy but imagine a patient who's using every last ounce of their histamine that they that they have in their brain and you give them this drug and you'll really throw them for a loop so be careful about these drugs be a little bit careful with prescription sleep aids and make sure that you're if if your doctor has never heard of dementia with Lewy bodies before then you might be a little bit careful about accepting a prescription for a sleep aid from that doctor go to some you know talk to somebody who knows what this illness is and you know get some reassurance that that sleep aid is not going to you know make them go crazy trouser is my favorite be cautious with some of the common ones like ambien and lunesta because they can sometimes make people act a little bit crazy old antidepressants like amitriptyline or elavil nortriptyline a lot of the antidepressants that were around before the 1980s can be dangerous for a patient with dementia with Lewy bodies for the same reason that an antihistamine can be dangerous it can cause a lot of confusion and worsen a lot of the cognitive symptoms drugs for Parkinson's disease now sometimes a patient might a patient with Parkinson's disease dementia for instance who gets the Parkinson's symptoms first their doctor may have started giving them a medicine like Sinemet to help them with their tremor or their gait problems or their stiffness or slowness patients might have been on Sinemet for a while or some of the other medicines that are often used for parkinson's disease but if you get Parkinson's disease dementia or dementia with Lewy bodies sometimes these drugs can actually cause the hallucinations are worse in the hallucinations and so some patients really you know need some of these drugs in order to have a good quality of life but it always has to be done with the knowledge that there's a balance too much of the drugs may exacerbate some of the cognitive problems there we go and the other point I wanted to make remember when I told you that neuroleptic drugs anti-psychotic drugs can can be a problem so I'm giving you that same message here so those those drugs are often used for hallucinations so antipsychotic are neuroleptic drugs and those can often make those parkinson's-like symptoms worse so we're in a little bit of a quandary aren't we the drugs that make the hallucinations better make the Parkinson's worse and the drugs that make the Parkinson's better make the hallucinations worse and so doctors are kind of stuck I tend to do as little prescribing as possible for a patient with dementia with Lewy bodies because I think at the end of the day less is more the fewer drugs you get somebody on the less chance there is of having a side effect but you know this there has to be a very individual law you have to have a good relationship with your neurologist and really have a heart-to-heart discussion on what for your loved one constitutes quality of life and you know what finding that right balance that right risk benefit ratio for drugs to make that quality of life as optimal as possible so what's left so cholinesterase drugs which include exelon aricept and rasa die these drugs were originally invented for Alzheimer's disease it turns out that they're way more effective actually in dementia with Lewy bodies than they are in Alzheimer's disease in fact if I'm wondering about the diagnosis in a patient and I give them a prescription for a receptor exelon and that patient comes back to me a week later and says wow that drug was great like you know my husband is like he's back to his old self then I scratched my head and I say I bet that was dementia with Lewy bodies because these drugs work by boosting the level of acetylcholine one of the chemicals in the brain that's responsible for keeping the lights on in your brain keeping you alert and focused and I think patients with dementia with Lewy bodies have a particular deficit of that particular kind more so than a patient with Alzheimer's disease and all these drugs do the same thing they help to improve they reduce confusion they improve your orientation and they even out some of those fluctuations that I mentioned antidepressant drugs so particularly in a patient who's suffering from a lot of anxiety some of these newer antidepressants can be quite helpful and can be safe Zola and lexapro are just examples of drugs that I think are usually good first choices and they can help to relieve some of the mood swings antipsychotics again I tell I've hammered this point pretty heavily with you use these with caution there are a few antipsychotic drugs that are a little bit less likely to cause worsening of the parkinsonian symptoms but you still have to be careful with them and the important point that I try to convey to caregivers is when you're picking drugs and picking medicines and figuring out what to do for a patient with dementia with Lewy bodies make sure that you're treating the patient okay the most of the time patients of mine with dementia with Lewy bodies who have flagrant visual hallucinations those hallucinations are not scary they don't bother me I'm having a party there's lots of people sitting around and I'm having a great time there's no reason to give a medicine to stop that if it's not bothersome to the patient it might be very distressful and very scary to the caregiver but once you get used to that if it's not bothering the patient don't drug the patient up to try to get rid of that stuff let them have their party it's okay and the same thing with stiffness and other parkinsonian signs if your loved one is able to get up and go for good walks and is able to you know do the things and on a daily basis that he or she needs to be able to do and it's not impacting their quality of life you don't need to you have a drug to reduce somebody's stiffness if it's not bothering them okay so so make sure that you're treating the patient and not just trying to make the patient look good for an outsider and here's the hard part now I have an easy job because I get to see my patients for 30 minutes or however long in my office and then I say goodbye to them and then we see each other in you know six months or something but you guys have to live with this illness this is the hard work but there are some things that you all know much more about than I do I'm an expert on the science and what the brains look like under the microscope and the drugs and that stuff but you guys are experts on the day to day caregiving issues and you all have discovered better than I do that having a calm environment lots of physical activity a good steady routine not too much changing and not too many trips are okay but not too many of them a good steady routine finding what triggers there are for agitation and trying to get rid of those triggers you guys are experts on on these sorts of things and these kinds of you know simple caregiving interventions are way more powerful than drugs are for making a patient's life higher quality more livable so just in the last couple of minutes I want to chat with you about some research and this is very disappointing and it's embarrassing to me as a scientist we have very few to know clinical trials actually I just went on clinical trials gov and searched for Lewy bodies and saw almost nothing so there are very few drugs that are being tested right now for patients with dementia with Lewy bodies this is going to change because there is more attention to this disease you all by being a part of this organization you guys are important advocates you need to let your Congress people know that this is an important disease and that research money needs to be sent for treating this disease you need to go out there in the community and participate in organizations like this and the many other organizations that raise awareness of this illness again it's it's awful that dementia with Lewy bodies that so few people have heard of it we need to change that we need to get out there in the community and make sure people have heard of this illness and we need more clinical trials to to to treat this research that I'm involved in and that I think is really exciting has to do with improving the ability of doctors to get the right diagnosis during life so that we don't have to wait for the autopsy now I mentioned that it doesn't make a whole lot of difference for the patient to get the diagnosis right because the treatments are the same the treatments are pretty straightforward but there is going to be a time when that's different for instance in the next five years I think it's likely that we'll get an fda-approved drug to treat Alzheimer's disease that actually slows Alzheimer's disease down we don't have a drug like that yet and some patients who look like they have dementia with Lewy bodies remember I told you that many of those patients have Alzheimer's like changes in their brain so for the subset of patients with dementia with Lewy bodies in whom their cognitive problems have more to do with Alzheimer's disease than with the actual Lewy body problem they might benefit from one of these new Alzheimer's drugs but first we have to be able to get the right diagnosis in life so we're doing a lot of research at Stanford that has to do with new brain imaging scans and other types of what I call biomarkers a biomarker is something that you can measure in a laboratory in order to get the diagnosis right and I think that will in these coming years we'll have better biomarkers for dementia with Lewy bodies and other ones for Alzheimer's disease so that we can understand a little bit better what the actual molecules are in the brain that's causing the member problems are the cognitive problems in your loved one and eventually it's slow-going but I'm optimistic of a future where we're going to have very specific drugs for one disease or the other and so that's the way I think the future is going thank you all very much and we have some time for questions this is a table thank you so much that was fascinating I I really learned a lot from that I was particularly taken with your remarks about how you take a drug for one symptom and it's antagonistic to another symptom you have such as the drugs for hallucination versus the drugs for movement because this this happened to my husband and with the seroquel and on and on but I have another question and that has to do with a lot of people in our group report that when Lewy body patients have surgery with general anesthesia they go over the cliff yep oh I'm I hear that now this is what happened to my husband he was supposed to live 10 or 15 years and within 16 months he was it's gone so as a general rule a patient with dementia with Lewy bodies is going to react if if an average person takes say I twelve hours to kind of get all those anesthetics out of the system and to kind of get back to a more or less normal thinking just multiply that by five or ten for a patient with dementia with Lewy bodies it just takes much much longer for any drug whether it's benadryl or whether it's general anesthesia any drug that has a cognitive side effect that causes a normal person to have a little bit of change in their thinking just multiply that by five or ten and that's how much it's going to affect a patient with dementia with Lewy bodies so very commonly an outpatient surgical procedure turns to an inpatient hospital stay and dementia with lewy bond really yes there was a related question about neuroleptics it's if you are damaged by them does this wash out or is this permanent that's a good question so for most patients the effects of antipsychotic or neuroleptic medicines do wash out now we all have read about the fact that some of the especially old fashioned antipsychotic drugs that are given to patients with schizophrenia for instance when you take those drugs for years and years there can be some irreversible symptoms but that's not typically and usually patients with dementia with Lewy bodies have never gotten those high of doses for that long at the time I say thank you there were a lot of questions about the confusion about diagnosis but I feel I got those mostly before you covered that topic so I'm going on to some more specific questions here and continue with the drugs when and why would an amend to be used to treat Lewy body so namenda is a drug that just like those other drugs I mentioned was invented for Alzheimer's disease the the three drugs that I showed up there aricept exelon and rasa dine all three of those boosts that one chemical acetylcholine in the brain so they all three of those drugs work by the same mechanism namenda isn't a family by itself and it's not absolutely clear how namenda works it doesn't do a whole lot in Alzheimer's disease in fact I don't prescribe it that often but when those other drugs don't works or their effect wears off sometimes I'll prescribe it and I get a little bit it's kind of like squeezing just a tiny bit more juice out of out of the brain that's there I don't really prescribe it for patients with dementia with Lewy bodies because I don't think it's that effective of a drug and my general philosophy with dlb patients is to prescribe as little as possible you are you aware of any work being done in using herbal preparations and there's Chinese medicine from the last session for treating some of the symptoms that's a great question and I get that a lot and also questions about vitamin supplements these are hard questions and for the most part the honest truth is that we don't know scientifically whether alternative therapies like Chinese medicines or vitamin pills or other supplements we don't know whether they're helpful or not I am skeptical as a scientist I'm skeptical but I also know that research trials often haven't been done my advice to you is to be careful don't overdo it both financially don't be pouring out a lot of money to a company who says that they've cured dlb and they show you a before-and-after YouTube video be skeptical consumers and be aware that people might be trying to hoodwink you and don't shell out a lot of money and also be careful because even vitamin pills have side effects especially for a patient with dementia with Lewy bodies so don't load on too many things and if you do so do one at a time and do small doses first to try and if there's side effects then stop it is the question so the question was are there any side effects from vitamins that are known and the answer is yes and it entirely depends on the particular vitamin vitamin e is an example so vitamin E ten years ago we thought it was the next best thing to slice bread doctors told you take more as much of it as you can it's an antioxidant it cures everything the NIH ran a clinical trial of vitamin E to try to prove what everybody knew that it did to try to prove that it does in fact reduce heart attacks and strokes and do you know what they found they had to stop the trial because there were more heart attacks in the group of people who took vitamin E compared to the placebo it did the opposite vitamin b6 causes a peripheral neuropathy like diabetes if you overdose on it there are some vitamins that you can overdose on for sure for most vitamins like b12 it just ends up going into the toilet I mean most of it honestly just goes through your body and never gets absorbed and probably doesn't cause a lot of harm except for the money you paid for it thank you there's a question about MRIs how much our frequency of MRI will affect the brain in terms of radiation to make the conduct current medical condition worse great questions so whereas cat-scans use x-ray radiation so a cat scan is a fancy kind of x-ray machine and MRI is fundamentally different and how it works and MRI uses a magnet and radio frequency so the same radio signals that's on your FM radio and on your cell phone so magnets and radio waves are not known to cause any change in the brain tissue and so there's no in MRIs have now been around for a couple decades or more and there's been no side effect ever identified in the you know hundreds of thousands or millions of people who have undergone MRI scans unless you have metal inside your body and that's an entirely different story if you have a pacemaker it is not safe for you to be anywhere near an MRI machine but but a human body with no metal inside of it there's no known side effect well that certainly is good news for your research yes in using MRI so it's the cat scans we have to watch out for that's where there's x-ray radiation and each of us can take a certain dose of x-ray radiation per year is that equal to the amount of what they say is background one year's worth of background radiation can you have more than that and it's still safe you can have more than that and it's still safe but it always has to be weighed against what's the purpose of this cat scan and how much information is it going to give my doctor that's a good question to ask okay I have a couple of questions relating to symptoms our severe night sweats a symptom of sleep disorder in Lewy body so the answer is it can be although if a patient came to me when it was complaining of night sweats I would send them to their primary care doctor and to make sure that it's not something more common you know when a doctor hears about night sweats you think about certain infections like tuberculosis and other kinds of infections you think about blood cancers like leukemia and lymphoma you know people get night sweats for a wide variety of reasons and it's good to check out those other things to make sure it's not something else before you blame dementia with Lewy bodies for it okay are there any postural patterns that can be typical for people with Lewy body so unlike PSP progressive supranuclear palsy which has a very characteristic kind of hyper-extended posture it's a little bit different for dementia with Lewy bodies my patients range anywhere from standing you know pretty straight to having a stupid forward posture like a patient with Parkinson's disease has so I see I see a spectrum there okay this question is my husband also experienced big drops in blood pressure when his heart or prostate medications were changed the question is well alternatively he doesn't seem to show any change or improvement from taking cinemates should he continue taking it so let me answer the first part first patients with dementia with Lewy bodies their blood pressure can fluctuate so it can go from being high to low kind of unpredictably and sometimes medicines will affect their their blood pressure more than they might affect a normal person and so it does you do sometimes have to be careful and the thing you worry about is the blood pressure going low enough that you pass out so it's important to kind of monitor them have a blood pressure cuff at home perhaps and monitor the blood pressure and make sure that when you do change the medicine that it's not causing you know deep dips in the in the blood pressure and remind me what the second part was um he doesn't show any change or improvement on cinnamon right okay so sometimes patients with dementia with Lewy bodies will you know again parkinsonian symptoms are common tremor stiffness slowness balance problems and sometimes Sinemet can help with those symptoms and sometimes it doesn't it's difficult to predict an individual patient but you have to again you have to have a good working relationship with your neurologist and if something's not working bring it to their attention and if it's not working then you ought to stop it okay a couple more general questions here is dementia always present or expressed with Lewy body my father's brain autopsy showed Lewy bodies but he never showed any dementia only REM sleep disorder and PD symptoms that's a wonderful question and it's a great example of how the what you see at autopsy doesn't actually differentiate between Parkinson's disease Parkinson's disease dementia and dementia with Lewy bodies these are all on a spectrum and they all look the same under the microscope so a pathologist who's never met the patient can't just look at the brain and know whether there were dementia symptoms or not the pathologist has no idea and can't tell the difference in how the brain actually looks and so that sounds entirely plausible that the brain looked like Lewy body disease but the symptoms weren't actually there yet maybe if that individual had lived another 10 or 20 years the symptoms would have come out another part to that was can Lewy bodies be present in the brain for a time before the dementia is noticeable that's another great question and the answer is yes the human brain is remarkably robust in fact in the substantia which is the part of the brain where Parkinson's disease lives and that's the part of the brain that's responsible when it goes bad for getting tremors and all those other parkinsonian symptoms it's not until 90% of the substantia is gone that you start to get the faintest wisp of a symptom 90% is gone by the time you have the faintest whisper of the symptom same is true in Alzheimer's disease all by the time a patient the first time a patient can't remember where they put their keys Alzheimer's disease has taken deep root in the brain has probably been there for at least 10 years and as you know the brain looks like a war zone by that point so the brain is very robust and it takes a lot of damage before you start to show symptoms this is a big problem when it comes to treatment I have a genetic question for you my husband is an identical twin two years ago he was diagnosed with Lewy bodies or dementia with Lewy bodies at the same time his twin was diagnosed with Parkinson's could this be a hereditary situation yes the answer is yes and for most patients it's not the case that you directly inherit dementia with Lewy bodies or Parkinson's disease from your mother or father there are some families where there is a bit of DNA that you inherit that that directly gives you the illness for most patients it's rather a whole genetic environment it's a combination of many genes that you inherit that influences your risk for getting the illness and so in an identical twin where the DNA is exactly the same there they share they share risk and it's interesting that one turned out to get Parkinson's symptoms first and the other one got cognitive symptoms first again I bet the pathologist couldn't tell the difference between their brains how do you explain how their symptoms differ this is part of what's fascinating to me as a scientist but it's really not known wow I'm getting some more specific questions here you said REM sleep disorder eventually would develop a person who has that would eventually develop DL B if they live long enough my son is six years old he has a sleepwalking problem his doctor said it would go away when he grew older should I be worried no sleepwalking is different beep walking does not occur during REM sleep sleepwalking occurs during deep sleep the EEG looks completely different it's a different disorder sleepwalking is fairly common it's also fascinating but it's a different illness and it's and it's not associated with any risk for Parkinson's or Lewy body disease you cited a problem earlier but an example of a person on an airplane and this this question hits me at home too do you think that the diminished level of oxygen in an airplane cabin and altitude causes the increase in confusion yeah certainly I think that's a great idea I think that's likely how do you think we're more susceptible to it when we have Lewy body yeah quite likely how common is it for dlb patients to not recognize a family member as being a family member example my dad seemed to recognize mom but he would say to her Zella really made a good dinner tonight as if he weren't talking to her one of the fundamental problems in dementia with Lewy bodies has to do with a part of the brain that puts together visual information okay so sometimes patients with DL beef they one of their first problems is that they don't think their eyes are working right and they may start to go to eye doctors before they go to a neurologist and the eye doctor keeps telling them where your eyes look fine I can't see any problem with your eyes so I think that there can be a problem in looking at a face and putting together those eyes and that nose and that mouth and comparing that template against your brains image of what my wife looks like and and making that comparison and say and making that positive identification this is my wife and I think it's that same part of the brain where the visual hallucinations come from the visual parts of the brain are playing tricks on you very often patients with dementia with Lewy bodies have trouble recognizing people there's a interesting syndrome where they can say you look like my wife but you're not my wife where did you put my wife you better bring her back I'm really upset very very strange behavior but I think that fundamentally it's due to an impairment in their brains ability to match those visual stimuli recognizing a house it's so common for a patient with dementia with Lewy bodies to be sitting in their living room saying take me home I want to go home why is all my furniture here this is not this is not the right place I want to go home and their loved ones are exasperated trying to you know we are home this is your furniture it's your living room they but it's that visual system that just is giving them conflicting information and their brain is just can't put it all together yeah I have a couple of questions about what caregivers specifically should do in certain symptomatic situations one is if a person is having a hallucination do you try to bring them back to reality or go along with them it depends I think that you probably know better than I do that fighting with somebody who has who's having an active hallucination is a losing battle you're going to lose that fight in most circumstances you're going to lose that fight and sometimes it's the better part of valor too to go along with it to provide a calming comment and you know to play along to the degree that it's able to calm your loved one and make them feel content because at the end of the day that's what's important I have a question here about exercises to help patients whose heads tilt forward or to the side can anything be done about that can you know can a caregiver do something above that yeah I think that I mean exercise in general is absolutely required for any patient with a cognitive problem especially dementia with Lewy bodies and I think that you know working on posture perhaps visiting with a physical therapist might make sense in a situation like that physical therapists are very good at suggesting very specific exercises tailored to an individual's posture problems aimed at strengthening certain muscles and reminding a patient to you know you know every 30 seconds or something think about their posture and correct it so a visit to a physical therapist might make sense in that situation okay a change of pace here I have heard and I suspect most people have that people with Lewy body don't die of Lewy body they die of something else is that mostly true is that and I think in our group I this is actually a question from Robin it's primarily either pneumonia or through stopping nutrition hydration that's a great question and it's a it's a hard one it's going to ask yeah it's a hard one to ask you know what gets written under on the death certificate may have little to nothing to do with how a person actually expired so people people die of very simple things like their heart stopping or their lungs not working perhaps from aspirating perhaps from you know choking on saliva or food or something I mean those that's kind of the immediate thing that might cause a person to die but why do they have trouble with swallowing why do they have trouble with maintaining their secretions well that could have to do with their neurodegenerative illness and so it could be that at the root of it that the neurodegenerative illness is creating a situation that's making it you know you know sort of bringing about death through one of these other causes mm-hmm it varies so much from person to person you know and we all can get a heart attack whether or not we have dementia with Lewy bodies or not we can all get cancer whether we so people die of a wide variety of things as they as they age sometimes it has to do with the neurodegenerative illness and sometimes not I think knowing that helped reduce my fear of the Lewy body disease itself knowing that that was something you had to learn to live with in some ways this final question actually goes back to the science we've talked a lot about dopamine levels and various other levels and the difficulty regulating levels this question is can neuroscience accurately measure amounts of dopamine serotonin and so forth so that you can use this information in prescribing so the answer is that it's complicated and I think this is symbolized by this tension between the Sinemet type medicines that treat Parkinson's and the Neurosci cut the the antipsychotic medicines that treat hallucinations both of these classes of drugs deal with dopamine Sinemet boosts up dopamine levels and the antipsychotics push it down and you can see how there's a tension some of the symptoms are due to too much dopamine and some of them are due to too little dopamine and it all has to do with exactly what part of the brain you happen to be in and that's why the medicines which go all over the brain don't work so well because they don't target just one specific place we don't have a way of measuring a dopamine level right in a specific part of the basal ganglia we don't have a way of doing that well except for that remember that brain imaging I showed you that dopamine transporter PET scan that I showed you that is a way that you can actually that doesn't actually measure the dopamine itself it measures the level of a protein that regulates dopamine but it can give you some sense of the regional concentration of it so we're getting there we're not quite there yet but we're working on it good to hear that good to hear someone like you really cares this is our final question and we're back to symptoms are there any ocular symptoms that occur with dlb for example tearing or watery eyes yes so there's a whole collection of symptoms that I call autonomic symptoms autonomic the autonomic nervous system is what controls sweating blood pressure urination all these kinds of automatic type type aspects of our of our wiring and our autonomic system can become symptomatic in Parkinson's disease and dementia with Lewy bodies so that abnormal sweating patterns problems with keeping the blood pressure regulated all of those can arise yes well I thank you all for your wonderful questions and thank dr. Kirchner for his wonderful song the preceding program is copyrighted by the Board of Trustees of the Leland Stanford junior University please visit us at med.stanford.edu

Channel: Stanford APDA

Views: 144,078

Keywords: Parkinsons, Parkinson's, Symposium, Conference

Id: UGtcvz2yT8M

Length: 69min 52sec (4192 seconds)

Published: Sat Mar 30 2013