In June 2021, Brain Support Network (BSN) was proud to co-host a panel discussion with Family Caregiver Alliance (FCA) about Lewy body dementia (LBD). The panel discussion followed the showing of the film “SPARK: Robin Williams and his Battle with Lewy Body Dementia,” produced by the Lewy Body Dementia Association (LBDA). Many such panel discussions were held over the summer 2021.
One unique aspect of FCA’s and BSN’s panel discussion on June 30th is that it featured two caregivers (Dianne and Denise) to those with Lewy body dementia, where the LBD diagnosis was confirmed through brain donation. Additionally, the panel discussion included Dr. Bruce Miller of UCSF’s Memory & Aging Center, who was prominently featured in the film.
The film delved into the known science and research of the disease, getting a diagnosis, and the impacts to the person with LBD and their family caregiver. Our panelists discussed:
- The confusing terminology — Lewy body dementia, diffuse Lewy body disease, Parkinson’s disease, dementia with Lewy bodies
- What percentage of people with Parkinson’s Disease go on to develop Lewy body dementia?
- Why was it hard for Robin Williams to receive a diagnosis of Lewy body dementia (while he was alive)? And what’s being done to educate physicians?
- Process of getting a diagnosis, the value in getting a diagnosis, the difficulty of getting a diagnosis when there are only psychiatric symptoms, family members’ reactions to the diagnosis, and the process of meeting with physicians
- Treatments for Lewy body dementia
- Symptoms of LBD including REM sleep behavior disorder, delusions, and fluctuating cognition
- Brain donation
- Optimism due to ongoing research
Both LBD caregivers mentioned the value of joining a local support group. We encourage everyone to find a local support group near you – whether it be for Lewy body dementia or Parkinson’s disease. One of the caregivers even participated in an Alzheimer’s disease group. You can find a list of LBD support groups here.
After the film and panel discussion, Family Caregiver Alliance (caregiver.org) shared an LBD resource list with attendees. Those resources included:
- Family Caregiver Alliance’s webpage on Dementia with Lewy bodies, which is a downloadable fact sheet
- Brain Support Network’s webpage on Lewy body dementia, including a downloadable list of “Top Resources on LBD”
- Lewy Body Dementia Association’s webpage for those living with Lewy
Additionally, to learn about brain donation for LBD, Parkinson’s, and other disorders, contact Brain Support Network.
If you live in Northern or Central California, contact Brain Support Network about joining our local LBD caregiver-only support group meetings.
The panel discussion was approximately 40 minutes long and can be viewed here:
One of Brain Support Network’s volunteers, Monica Monack, took notes on the panel discussion. See her notes below.
Lewy Body Dementia Panel Discussion Following the Movie “SPARK” – Notes
Hosted by Family Caregiver Alliance and Brain Support Network
June 30, 2021
Summary by Monica Monack, Brain Support Network volunteer
Bruce Miller, MD, behavioral neurologist, UCSF Memory & Aging Center
Dianne Weitzel, leader of Brain Support Network’s LBD caregiver support group
Denise Dagan, volunteer with Brain Support Network’s LBD caregiver support group
Robin Riddle, CEO, Brain Support Network (brainsupportnetwork.org)
Robin: It’s a delight to be here with all of you and to be sharing this wonderful film and our terrific panel.
So let’s start off with Dr. Miller. The terminology is incredibly confusing. We have Lewy body dementia, diffuse Lewy body disease, Parkinson’s Disease, dementia with Lewy bod. Can you help demystify this?
Dr. Miller: Let me give you a slightly complicated answer, because I think this is really an important question for families. So, this used to be Parkinson’s Disease and we used to think of Parkinson’s Disease as a movement disorder that had no effect on behavior, mood, anxiety or emotion. And, over time we learned that many people with Parkinson’s Disease don’t actually present to the family, to the doctor or to society with a change in movement. Rather they present with changes in things like visual hallucinations and delusions and profound disorders of sleep. In fact, I would argue that most people with Parkinson’s Disease have a propensity to these problems. Although some may present with a pure movement disorder, these are different parts of the elephant. The elephant is the bad protein that aggregates in the brain. It’s called alpha synuclein and it causes Parkinson’s Disease, Lewy body dementia, and dementia with Lewy bodies. They’re just different ways that the same molecule can wreak havoc in the brain. I think as we move toward therapies, what’s going to be incredibly important is to recognize all the different forms, with the one common molecule which is alpha synuclein. And I believe that in all of our lifetimes there will be a day when someone presents to the doctor with a sleep disorder or the psychiatric manifestations or the cognitive manifestations that we call Parkinson’s with dementia, the treatments will be focused on the primary cause which is this mis-folded protein.
Robin: One of the questions that some [of today’s] attendees asked is — what percentage of people with Parkinson’s disease go on to develop Lewy body dementia?
Dr. Miller: A really great study done in Norway suggested that at least a quarter of people with Parkinson’s Disease, if you look carefully, also show cognitive or behavioral issues early. I think the field has ignored this a lot. They focus on the Parkinson’s and forget that the severe anxiety, which is the same disease, is so disabling to loved ones and caregivers. I would say in a very high percentage of people with Parkinson’s it’s not just a movement disorder. It doesn’t start in the movement part of the brain. It starts in the part that is involved in regulating emotion, attention, sleep. These symptoms are often there with the Parkinsonism. So, very common.
Robin: It seems odd then that it was so hard for Robin Williams to ever receive a diagnosis of Lewy body dementia. He had access to all these resources, different kinds of resources, and yet he was still unable to get a diagnosis. Why was that?
Dr. Miller: Realizing that the mood disorder and the anxiety disorder are not just late life psychological problems, adjustment disorders. This is a disease. This is the beginning of Lewy body dementia. Until we realize this we will misdiagnose and, even more tragically, we will treat them inappropriately. Sadly, I think Robin got a lot of inappropriate treatments that didn’t focus on the biology of his problem. It had a real chemical basis, a real biological basis, but people didn’t really grapple with that.
Robin: I’d like to turn now to one of our caregivers.
Dianne, what was the process like for your husband getting diagnosed? Was it a challenge or was it fairly straightforward?
Dianne: It was definitely a challenge. We were living in Hawaii. I noticed first off that we were not on the same page anymore. Things that I would tell him, he would say he hadn’t heard before. When we had appointments, he didn’t register that. There were so many things. He forgot where we were going. He didn’t know where he was going sometimes. He didn’t recognize friends that we knew he should know. His doctor on the island doctor said there’s nothing wrong with him. When a farm and garden owner called me to tell me Gary had just been there and picked up some things, he said he was afraid that Gary wouldn’t make it home because he was driving by himself. I called the doctor immediately and the doctor said oh yes we will do a check on that. Bring him in first thing in the morning. We did that and later that day he said there’s nothing wrong, that his blood tests are totally normal. My friends were saying what’s wrong with Gary? He has no expression through his face. It’s like a mask. I’d try to take a photo and say smile Gary and he’d say I am smiling. Finally it was a urology friend who lives in San Francisco, that we saw on a trip back, who said what’s wrong with Gary? Please, I asked our friend, help me if you can and that’s when we saw doctors in UCSF. They told us he doesn’t have Parkinson’s, which is what our friend thought. They thought Gary had dementia with Lewy bodies.
Robin: Dianne, could you talk a little bit about your husband’s response to the diagnosis? Did he accept it? Was he in denial about it?
Dianne: He was very, very relieved when the neurologist told him he didn’t have Parkinson’s. And then he just turned off. He was fine with that diagnosis. He can still drive, he can do whatever he wants to. He’s not got Parkinson’s. But the UCSF doctor said you have either Lewy bodies or multiple system atrophy. I had him write those down. I went onto the web and researched both of those and subsequently we were given some medication to see if it will help. It was amazing how his expressions came back to his face he was able to show his emotions. A lot of things were working again after that medication. I was relieved but things got worse later on. There are always new medications and we knew what the diagnosis probably was. Even though, you can’t tell for sure until an autopsy.
Robin: Denise, what did it mean for your family to receive a diagnosis and was your dad accepting of it?
Denise: My dad was diagnosed with Parkinson’s Disease. He was resistant to get the diagnosis because he was walking like Tim Conway, you know when he did the old man with the shuffling feet and not going anywhere. We encouraged him to find out if this was just old age or something else. But he had a trip planned for a christening of his first grandchild on the East Coast and he didn’t want any doctor telling him couldn’t fly. When he came back, we went to the Parkinson’s Institute because he confessed that his doctor suggested he see a neurologist. When we got the diagnosis and the prescription for Sinemet, the first pill turned back the clock by two years. It was amazing and he was very pleased because he wasn’t struggling any longer. He had been treated for Parkinson’s for about five or six years before he had his first hallucination. We learned a lot through the Parkinson’s phase. That the more you know, the more you can help yourself. So when the diagnosis changed, we kept pursuing information on Lewy body. At the time, it was probably 15 years ago, neurologists were still expanding their knowledge of Lewy body so there was nothing on the web. I think there was one book out.
Robin: What about the process for meeting with physicians? Did you always have another family member present besides your father? How did you get information?
Denise: Dad had an excellent neurologist in Corvallis, Oregon. She actually knew what Lewy body was when we suggested to look into this. It was after that, that my mom needed hip replacement and they moved in with us to have the surgery at Stanford. They lived with us for the better part of two years and I went to the neurology appointments with him at Palo Alto Medical Foundation. I started to learn a lot about what he needed, such as adding another dose of Sinemet or a time release at bedtime. But when they moved back to Oregon, I wasn’t involved anymore. After some time, we had to move them to California so they could get more support.
Robin: Dr. Miller, we’re receiving a lot of questions [from today’s attendees] about treatment. Both Dianne and Denise mentioned some dopaminergic therapy that’s very common for Parkinson’s Disease. Could you speak to the treatments for Lewy body dementia and also the conundrum associated with treating psychiatric symptoms vs movement symptoms?
Dr. Miller: I think of three neurotransmitters. There’s the dopamine, and that’s involved with movement and some attention. Then there’s serotonin, which is where we see so much depression, as an early feature of this. Sometimes unalleviated anxiety in someone who was never anxious. I think of that aspect of the disease as a separate but treatable component. The third part is attention, and sometimes there’s a profound loss of acetylcholine. I think if we can boost acetylcholine we can diminish the hallucinations, trouble with attention, and tendency toward becoming delirious. We know that in people with Lewy body, because they’re so deficient in acetylcholine, if a little thing happens like a urinary tract infection or a post-operative, this cholinergic deficiency leads to a profound delirious state and alterations in sleep and wake. I often treat all three in different stages of the illness. And we can usually make a significant impact on caregiver’s and patient’s lives by thinking about these different treatable pieces. Your advocacy work is so important in telling families to not accept it’s untreatable. It’s uncurable now, but it’s very treatable.
Robin: Since it is uncurable, what is the value in getting a diagnosis?
Dr. Miller: The value is helping the people understand why and what. Most of us feel very poorly with mystery in our own lives. Having certainty about what happened is important. Because we have our own unique therapies for Lewy body, it’s important to diagnosis this properly. It’s always important for me to explain to families, I know this person is different. But it’s because there’s a chemical change. Better understanding of the architecture of this is really important. I’m an optimist and I believe we are on the verge of disease modifying therapies for Alzheimer’s, frontotemporal dementia and Parkinson’s. We have huge support through various foundations, like the National Institutes of Health, and we understand so much more. I think we’re really going to cure these diseases. So this is why I think diagnosis is so key.
Dianne: I think another reason for the diagnosis being important is that my husband had to have knee surgery halfway through his illness and the type of medications used in surgeries can have a big part on what the brain does. During surgery they did a saddle block on him rather than the normal anesthesia. But after surgery he went into a rehab facility where he was given the totally wrong kind of medication. That doctor didn’t know they couldn’t give that med to him. He was totally anxious and was having the most horrible hallucinations you could possibly imaging.
Dr. Miller: It’s such a great point. That’s preventable. Avoidable.
Robin: Denise, what kind of psychological or behavioral issues did your father exhibit during his course and what was challenging for you?
Denise: He had REM sleep disorder for at least a decade, probably longer, prior to his movement challenges. To the point where he threw himself out of the bed into the corner of the room behind the nightstand one evening when I was staying with my parents. We just assumed that because he was in WWII that these were sort of PTSD nightmares. Then you learn more and find out that moving and talking in your sleep nightly is a very common experience. And that you’re much more likely to develop Parkinson’s and Lewy body dementia. I don’t know how Mom continued sleeping with him in a double sized bed for as long as she did. I’ve wondered if her lack of sleep contributed to her early on-set of Alzheimer’s. As for hallucinations, the first time that happened to Dad was when he had taken an over the counter medication, something for muscle cramping or a cold. We had called the neurologist and she said to keep him comfortable and it’s fine if he takes this medication. Loosely translated it means you’re going to have a very interesting afternoon. He saw neighbors from Oregon driving past the house in California, he saw a woman nursing a baby in our patio. It was wild. Trying to keep him in the house was really challenging. They became more frequent as the disease progressed. At one point the neighbors found him out by the highway and brought him home. It was some effort to keep him safe. He wanted to dismantle the lawnmower. He wanted to work with saws in his shop. We were really afraid he would injure himself. But my mom’s feeling was that this is how he lived before he got sick. And if it’s what kills him, he would rather die doing what he loved to do. Even if he didn’t know what he was doing. It was scary.
Robin: Dr. Miller, Denise brings up REM sleep disorder. This also came out in the SPARK film in terms of Robin Williams suffering from some sleep issues. How common is REM sleep behavior disorder in Lewy body dementia?
Dr. Miller: It’s still under active study, but I would say somewhere between 60% and 80% of people with Parkinson’s had either a prodrome before the Parkinson’s REM behavior or it develops soon after the diagnosis. It’s really common. Thinking a few years down the line, if we have a great prevention for Parkinson’s and we knew it was beginning, that we would be able to intervene before any other problems occurred. The sleep disorder is really the beginning of Lewy body dementia. It may turn out to be a fortunate feature of the illness that we get sleep changes that tell us that Parkinson’s is coming.
Robin: Dr. Miller, back to your mention of movement symptoms vs psychiatric symptoms. One of today’s attendees mentioned that her mother had hallucinations as her primary symptom. I would imagine that having a psychiatric symptom like that would be very confusing for neurologists.
Dr. Miller: There are some features that Denise described really well that should have you think about Lewy body. The hallucinations tend to be formed as little children, little people, and very vivid scenes. Sometimes very disturbing to the person, but sometimes they actually sit back and enjoy them. So hearing that anyone later in life is getting hallucinations, we want our physicians, whether they are in primary care, the ER, or neuro and psych, to say this is something more than schizophrenia, which of course doesn’t happen later in life. I think the problem is education. I think that’s where this breaks down.
Robin: What is being done about educating physicians?
Dr. Miller: We’re just beginning. And part of the problem for some of this is that primary care docs don’t get reimbursed for spending a lot of time with families and thinking through symptoms like this. There is a program called ECHO that the state centers and Alzheimer’s centers all across California are beginning to work in. The program is opening up time for primary care physicians, and probably eventually families, to consult experts. I think the more and more we get palpable evidence that we have therapies for these disorders, families will demand that they get better diagnoses, care and treatment. I think this is coming and the work that you all do in advocating is going to change the way this is dealt with. The suffering that families go through with Lewy body is about as high as any illness that I have ever worked with.
Robin: Symptoms of LBD are hallucinations and delusions. Dianne, could you speak about your experience with Gary and delusions?
Dianne: He had a sundowning every afternoon that would go on for hours and hours. He was a pilot for a commercial airline and the first symptoms that we were really aware of appeared about 5 years after he retired. So at age 65 he was already showing some terrible signs. One was that every afternoon he kept reaching out with his fingers trying and to make his eyes focus to what he saw as the cockpit of the airplane. He knew he wasn’t seeing it properly and he couldn’t tell what his altitude was. He kept trying to make his eyes focus on that dial. Then he couldn’t remember where he was going and he would be terrified that he was about to cause an airplane with 200 passengers to crash because couldn’t he see well, he couldn’t land an airplane in that condition. When I would see him doing this I would to tell him it’s ok, that the co-pilot has landed your airplane and we’re sitting at crew rest. And he would be so relieved and say thank you for telling us, I’m so glad we’re here, now let’s get this thing on the ground! And it would start all over again. So, I would let him know that we’re on the ground and then have to change the whole focus of whatever we were doing. Change the subject really quickly before he gets back into the problem. It was difficult. Another part was later on, as caregivers, we became his jailers. He was also an Air Force pilot for 10 years and military training taught him what to do if they were captured. So we were his captors.
Robin: One of the other very challenging symptoms of Lewy body dementia that we hear from our local support group is fluctuating cognition. Where the person can seem totally fine one minute and not the next. Or it may very on the basis of an hour or day.
Denise, your father exhibited this symptom. Could you give an example of it?
Denise: I have such a good example of this. I came out from my bedroom one morning and the caregivers had gotten him dressed and he was sitting on the sofa in the living room. His shirt was tucked into his pants and it was really bunchy. I knew by the afternoon it was going to be irritating and he was going to be less cooperative and upset. So I asked to him to stand up and I’ll smooth out your shirt so you’ll be more comfortable. I got it straightened out and got him back on the couch when my mom came out and said breakfast was ready. I said, ok Dad let’s stand up and we’ll go have breakfast. He had no idea what I was talking about. It was like I was speaking Martian. Any suggestions I would make to use the arm of the sofa to get up, or his walker, or something to lever himself out of the chair was confusing to him. After about 5 minutes of this, I was at my wit’s end. I thought about Dad, and he’s always about his food, and said to him, Dad go eat. And he stood up and went into the kitchen because he wasn’t thinking about what I was trying to say. It’s just that fast…English to Martian to English in just 10-15 minutes time.
Robin: Dr. Miller, what’s the neurochemical or protein based explanation for fluctuating cognition?
Dr. Miller: This is acetylcholine, I believe, and we have what we call a cholinergic deficit in people with Parkinson’s, sometimes even before they develop symptoms. So, when we measure this we see a profound deficit with acetylcholine. That’s the alerting chemical. It’s part of our reticular activating system that says wake up, attend, focus. And when someone has symptoms like this, I always treat them with a cholinesterase inhibitor because it boosts acetylcholine. The FDA approved a rivastigmine patch, but I think there are other ways to do this. I think this is something again that we can really manage and help people with. We don’t get rid of the deficit, but we can biochemically alleviate some of the loss of attentional capability.
Robin: Denise, back to you, I’m wondering what were some helpful resources and information as a caregiver?
Denise: My parents were older. Dad was 80 something when he was diagnosed with Parkinson’s. They had a computer that they used mostly for email. So I would look up information as Mom or Dad complained about his challenges. I’d try to find some information about preventing falls or wandering or whatever. When a friend recommended a caregiver support group, I sought out a Parkinson’s support group. I found sharing information with other people and learning from other people about how they cope with different challenges to be immensely helpful.
Robin: Dianne, how about you? What did you find most helpful to you? What resources?
Dianne: I also went to whatever I could to find to help make him more comfortable. I went to Parkinson’s classes and support groups. I went to Alzheimer’s support groups. I took a week-long class with Family Care Alliance. And then just quite by accident, as I was getting a [fall] monitor, the installer mentioned Robin Riddle’s [local Northern California atypical parkinsonism] support group. That’s where I said to myself that they are actually “Parkinson’s plus” which is exactly what I need. That has been so helpful to me. It’s why 10 years later I’m still helping the group because they helped me. That is also why [I signed Gary up for a] brain donation and a total body autopsy.
Robin: Both of you donated your family member’s brains.
Denise, can you talk about brain donation and why you donated your father’s brain and what the value was?
Denise: Dad mentioned that he hoped there would be a cure for Parkinson’s before it got him. After I joined the [local Northern California Lewy body dementia] support group and learned about brain donation, I thought you can’t contribute to the cure if you’re not part of the research. So, we donated the brain without asking him. He’s part of the research now.
Robin: One last question to Dr. Miller. Could you say what is happening in the area of research and why you are so optimistic?
Dr. Miller: I think that we have a community that is very organized around cures. This is unbelievably important. Setting lofty goals. Regarding research funding, we are in a very good space. Over the last 5 years the National Institute on Aging went from less than a billion to $3.5 billion dollars a year. This is extraordinary. The new administration said they are going to get us even more funding. Also systematic efforts. Not so much in the pharma companies yet, but in the academic labs, to figure out ways to prevent bad proteins from aggregating, from decreasing the inflammation when the proteins aggregate, from telling the cells to figure out better ways of clearing out the bad proteins. This is a common thread of all degenerative diseases. As we get older we are not as good at getting rid of the garbage in the cell. Brilliant investigators, like Dr. Ana Maria Cuervo at Albert Einstein University, are designing drugs that will help the cell get rid of this bad protein, alpha synuclein. I think our diagnostic abilities are approving. It could be a lot better with Parkinson’s. We don’t have those great blood biomarkers yet that we do with Alzheimer’s. But they’re coming. Early diagnosis, early intervention, and smarter drugs hold a very optimistic picture.
Robin: That’s such good news.
Robin: Christina, we’re at the end of our time so I’ll turn things back over to you.
Christina [with Family Caregiver Alliance] Thank you so much to Robin, Denise, Dianne and Dr. Miller. Thank you to the Lewy Body Dementia Association for the use of the film. We hope that the film and this discussion has shed more light on this disease.