Episode 87: What is Lewy Body Dementia and How Does it Relate to Parkinson’s?

Dan Keller 0:08

Welcome to this episode of Substantial Matters: Life and Science of Parkinson's. I'm your host, Dan Keller. At the Parkinson's Foundation, we want all people with Parkinson's and their families to get the care and support they need. Better care starts with better research and leads to better lives. In this podcast series, we highlight the fruits of that research, the treatments and techniques that can help you live a better life now, as well as research that can bring a better tomorrow.

Besides the well-known non-motor and motor symptoms of Parkinson's disease, there is also a risk for developing dementia. It is estimated that about 40% of people with PD will develop dementia at some point, usually later in the disease. Some risk factors are older age, older age at disease onset, longer disease duration, and more severe motor symptoms, among others. Parkinson's disease dementia falls within an umbrella term of Lewy body dementia, along with another condition called, somewhat confusingly, dementia with Lewy bodies. Both forms of dementia are characterized by the accumulation of Lewy bodies within nerve cells in the brain. Lewy bodies are clumps of alpha-synuclein and other proteins and disrupt nerve cell function.

I asked Dr. Jennifer Goldman of the Shirley Ryan AbilityLab and Northwestern University in Chicago to help make sense of these two related forms of dementia, and to give people with PD some practical advice about things to be aware of in terms of their medications. Let's talk about Lewy body dementia and its related forms. What is it, and how does it present? How does anyone know that they have it, or that a person with Parkinson's may have it?

Dr. Jennifer Goldman 2:17

Lewy body dementia is a progressive brain disorder in which we see Lewy body deposits, which are protein deposits of alpha-synuclein, accumulate in different parts of the brain. And these parts of the brain that can contain Lewy bodies are those that are affected in cognition, behavior, and movement, as well as some other motor and non-motor features.

Dan Keller 2:47

There's various forms, it seems. There's Parkinson's disease dementia, there's Lewy body dementia, there's dementia with Lewy bodies. Can you sort these out, or at least for people with Parkinson's disease, how does that relate to them?

Dr. Jennifer Goldman 3:01

So actually, Lewy body dementia refers to an umbrella term that includes both Parkinson's disease dementia and dementia with Lewy bodies. The terminology can often be confusing to people seeking care, as well as people in the field, among healthcare professionals. There are several features that are shared between Parkinson's disease dementia and dementia with Lewy bodies, but there are also some differences between the two in terms of their course, some of the core features and their progression, and perhaps some of the ways we treat them.

Dan Keller 3:42

Since there may be some confusion, does this get either underdiagnosed or delayed diagnosis?

Dr. Jennifer Goldman 3:50

With the terminology confusion, certainly, there can be diagnostic confusion, and it's actually not uncommon that we see people seek out many different doctors before they get the right diagnosis. For example, people can have many different types of symptoms. In addition to motor Parkinson's, they can also have mood changes, such as depression or anxiety. They can have cognitive changes, hence the name dementia with Lewy bodies, when the cognitive changes occur early in the course, as well as autonomic changes affecting the gut system, bladder control, blood pressure, and so forth. And so sometimes people end up seeing multiple specialists before they end up seeing a neurologist or a movement disorder specialist or even a dementia specialist to get the right diagnosis.

Dan Keller 4:47

When would this occur in the course of Parkinson's disease, if it does occur?

Dr. Jennifer Goldman 4:52

So when we think of Parkinson's disease, we tend to think in the context of its motor symptoms, both historically and also even still to present day, but we recognize that cognitive changes can be part and parcel of Parkinson's. More commonly, early in the course of Parkinson's, we'll see mild changes in cognition, meaning slower thinking, slower processing, trouble with multitasking or organization, trouble with attention, and so forth, and those are often what we think about with mild cognitive impairment.

When we see more severe cognitive changes, and particularly when they affect someone's function or their ability to act independently in activities of daily living, or at work, or in their hobbies, we think of that being a form of dementia. So, the term we use for dementia represents cognitive changes that occur gradually, may affect multiple areas of cognition, or different cognitive domains, and affects someone's functional abilities.

The dementia that we see in Parkinson's very often has some similar features to the cognitive deficits we see when it's mild, such as those like attention problems or executive function, as well as even visual-spatial difficulties, perhaps also with memory, but not necessarily so, and this tends to occur later in the course of Parkinson's. So, after a good number of years, people will develop a dementia.

So, one of the distinguishing factors in the definition, or the terminology, which can sometimes be confusing for people, is that in Parkinson's disease dementia, the motor symptoms precede the dementia. In contrast, in dementia with Lewy bodies, the primary diagnostic symptom is a dementia syndrome. So, those cognitive changes are early, and motor features, if they are present—since not all dementia with Lewy body patients will have Parkinson's—occur either after the onset of dementia or somewhere concurrently.

Dan Keller 7:14

Is it treatable? It sounds like it's such a multisystem disease, it's going to be a complex regimen of treatments.

Dr. Jennifer Goldman 7:22

So both Parkinson's disease dementia and dementia with Lewy bodies, so under the umbrella of Lewy body dementia, have symptoms that can be treatable and managed. So right now for both of these conditions there unfortunately is no cure to date or any way that we definitively know can slow down or halt the progression. However, one way we think about treating these symptoms is by a variety of multi-pronged approaches. So medications to target specific symptoms, and then non-pharmacologic therapies to help augment the symptom management and the medications.

So for instance, cognition or dementia is a symptom and feature that we try to manage in a number of different ways. So there are some medications that have been studied for dementia in Lewy body disorders. For example, in Parkinson's disease dementia, there's one FDA-approved medication that is rivastigmine, for the indication of Parkinson's dementia. For dementia with Lewy bodies in Japan, a cousin to rivastigmine, called donepezil, is approved in their country, and these medicines have some modest effect, but are thought to help with symptoms such as attention, executive function, memory function, and so forth.

In addition to medication therapies for managing cognitive changes, it's also important to think about what else we can do for them, which could involve a growing role for physical activity, physical exercise, cognitive exercise, or cognitive training, compensatory strategies to deal with changes in thinking and memory, as well as helping the care partner with the person who's having the cognitive changes.

Dan Keller 9:24

Is there any problem with drug interaction that you would use for dementia with Lewy bodies versus the initial presentation with Parkinson's disease? Any competition or things to watch out for?

Dr. Jennifer Goldman 9:38

So, there are a number of things to watch out for. So some of the other symptoms that both Parkinson's disease dementia and dementia with Lewy bodies can share include some behavioral phenomenon, such as psychosis—so hallucinations, particularly visual hallucinations, or delusions, such as paranoia or infidelity—as well as other features like REM sleep behavior disorder, where people may act out their dreams when they're supposed to be still and asleep, and even autonomic changes like orthostatic hypotension or low blood pressure, or drops in blood pressure when people stand up. And so those are some other symptoms that we work to manage, both with medications or with non-pharmacologic therapies.

One word of caution, and particularly with people who have dementia with Lewy bodies, is that the medicines we use to treat the motor symptoms that have dopaminergic qualities can be a bit harder to tolerate because side effects can be more prominent or more likely to occur, such that doses of the Parkinson's medicines, the dopaminergic medicines in those patients, may exacerbate or bring on hallucinations. So we need to be careful of the dose, usually with the message, if one is going to treat the motor symptoms with dopaminergic medication, to go with a low dose and titrate it slowly with careful monitoring.

There are some medicines that can be fraught with difficulty with dementia with Lewy body patients, because patients can be very sensitive to a certain class of medications, such as neuroleptics. And we see this a little bit less now with newer types of medicines or antipsychotics for treating the hallucinations and psychotic symptoms, but patients can be very sensitive to them and have marked motor complications and mobility issues, and really profound changes in their level of function.

Dan Keller 11:49

Are there any cautions if someone is being treated for this when they enter a medical setting, especially things like surgery, anesthesia, someone who's going to prescribe a new medication? How should they coordinate their care?

Dr. Jennifer Goldman 12:05

For Lewy body dementias in general, also including Parkinson's disease without cognitive changes or without dementia, it's very, very important to have a clear plan for hospitalization and clear coordination among the healthcare professionals on a team, the patient, their care partner, and family. There are some medicines that people with Lewy body disorder should not receive, and these are medicines that block dopamine. Some of these can be medicines for nausea, called antiemetics, that block dopamine. Some of them can be psychiatric medicines, most commonly used to treat psychosis, such as the hallucinations, and the older antipsychotics, and they can aggravate Parkinson's, because basically they block dopamine and counteract all the medicines we would be using to treat the motor symptoms.

So it's very important for people with Lewy body disorders, with or without any cognitive changes, to be aware of this potential when there's surgery or hospitalization, and it's often important and useful for people to carry a card in their wallet stating this, or to have this in their medical record, and to have this available, whether it's in a kit, like the Aware in Care kit from the Parkinson's Foundation, to help prevent some of these complications in hospitalization.

Dan Keller 13:34

Is there active research in this area?

Dr. Jennifer Goldman 13:37

There is quite a bit of research going on in Lewy body dementias, all the way from trying to understand why it occurs, and whether there are differences between Parkinson's disease dementia and dementia with Lewy bodies, despite the fact that they share many clinical symptoms, and even pathology when we look at brain tissue or microscopic tissue or biomarkers, to research that's involved in trying to understand how can we predict or detect early changes in people who might go on to develop Lewy body dementia, all the way to treatments. And those treatments actually include medications that are under study for various symptoms, including cognition or visual hallucinations, as well as even some newer trials that are starting to look at the role of physical exercise, cognitive exercises, and even neuromodulation with things like deep brain stimulation or transcranial magnetic stimulation.

Dan Keller 14:42

What have we missed, or is important to add?

Dr. Jennifer Goldman 14:44

So, I think one aspect to think about in terms of dementia with Lewy bodies as a form of Lewy body dementia is that it is actually quite common. It is the second most common dementia next to Alzheimer's disease. It's thought to affect 1.4 million individuals in the United States, and in a way is the most common dementia that no one's ever heard of, or fewer people have heard of. And I think with greater education and awareness of Lewy body dementias at large, including Parkinson's disease, we can start to change that landscape in a way.

Dementia with Lewy bodies is caught in a cross between Alzheimer's disease and Parkinson's disease, and I think for that reason, as well as the many different symptoms that people can present with—from sleep disorder, hallucinations, Parkinson's, autonomic features, along with the cognitive changes—can make it very confusing to find where to attain help and get a diagnosis.

Dan Keller 15:56

Is there a take-home message for someone who may have some dementia, but at this point it really hasn't been nailed down?

Dr. Jennifer Goldman 16:04

So, one take-home message would be to continue to seek the answers and seek out a specialist to help identify the cause of the cognitive symptoms, the cause of the dementia, whether that might be with clinical features or certain types of brain scans or other testing that might help pinpoint the etiology or the cause of the particular dementia. And that's important because it can change management, it can change what we think about prognosis, as well as eligibility for different research studies, and so it's just important for people to have the best understanding of their disease that they face and be able to have the best management, so they can have the best outcomes associated.

Dan Keller 16:53

Great, thank you. You can visit parkinson.org/dementias to learn more about the types and symptoms of dementia, along with treatments and frequently asked questions. For a good discussion of dementia and the differences between Parkinson's disease dementia and other forms of dementia, including dementia with Lewy bodies, see our expert briefing with Dr. Goldman from 2016. She talks about why dementias occur, possible risk factors for Parkinson's disease dementia, early signs and markers for it, management strategies, and practical tips for people with PD and their care partners.

To see it, go to parkinson.org/ebs, click on past expert briefings webinars in the left column, and scroll down to the title Dealing with Dementia in PD. Dr. Goldman also headed up a working group on maintaining cognitive function, and you can see some of its recommendations by searching the website on her name, Goldman, to see the article titled Tips from the Pros: Maintaining Cognitive Brain Health in Parkinson's Disease. And in episode 27 of this podcast series, she addressed cognitive and behavioral challenges in caring for PD.

And finally, mark your calendar for a new expert briefing series launching this September, which includes a webinar with Dr. Gregory Pontone called Mental Well-being and Memory. You can register for our expert briefing series by visiting parkinson.org/ebs.

As Dr. Goldman recommended, because of potential medication interactions in people with Lewy body disorders and PD in general, people should keep a list of medications they're taking so that they can present it in any healthcare setting. She specifically mentioned the Parkinson's Foundation's Aware in Care kit with tools to help keep people with Parkinson's safe in the hospital. That can be downloaded or requested by visiting parkinson.org/awareincare, and you'll see the kit featured there, or you can call our helpline, where our PD information specialists can order a kit for you, as well as answer questions and provide information in English or Spanish about today's topic, or anything else having to do with Parkinson's.

You can reach them at 1-800-4PD-INFO. To receive news and updates about future events and resources, you can opt into our email list at the bottom of our website's homepage. If you have questions or want to leave feedback on this podcast or any other subject, you can do it at parkinson.org/feedback, or if you prefer, email us at podcast@parkinson.org.

If you enjoyed this podcast, be sure to subscribe and rate and review the series on Apple Podcasts or wherever you get your podcasts. At the Parkinson's Foundation, our mission is to help every person diagnosed with Parkinson's live the best possible life today. To that end, we'll be bringing you a new episode in this podcast series every other week. Until then, for more information and resources, visit parkinson.org or call our toll-free helpline at 1-800-4PD-INFO, that's 1-800-473-4636. This episode is supported by a grant from Genentech, a member of the Roche Group. Thank you for listening.

Jennifer G. Goldman, MD, MS is the Section Chief for Parkinson’s Disease (PD) and Movement Disorders at the Shirley Ryan AbilityLab (formerly the Rehabilitation Institute of Chicago) and Professor of Physical Medicine and Rehabilitation and Neurology at Northwestern University Feinberg School of Medicine. Dr. Goldman is a Movement Disorders neurologist with board certification in Behavioral Neurology and Neuropsychiatry. She graduated from Princeton University magna cum laude and completed her MD at Northwestern University Medical School, Neurology residency at Washington University in St. Louis, and Movement Disorder fellowship and Master of Science in Clinical Research at Rush University in Chicago. Dr. Goldman is a clinician-researcher who has been a pioneer in the cognitive and behavioral aspects of PD and movement disorders and a longstanding champion and leader in interdisciplinary care.

As a clinician, she treats patients with PD, atypical parkinsonian disorders, dementia with Lewy bodies, dystonia, Huntington’s disease, and other movement-related conditions. She has implemented novel interdisciplinary, comprehensive care models for PD and movement disorders in both neurology and rehabilitation settings. Her research focuses on advancing our understanding of the non-motor and motor features of PD and movement disorders, using neuroimaging and biomarkers, and on developing pharmacological and non-pharmacological interventions to improve or prevent these symptoms. Dr. Goldman has been funded by NIH, Michael J. Fox Foundation, Parkinson’s Foundation, among others and has published over 85 research articles and book chapters.

As a clinician, Dr. Goldman is passionate about offering a holistic, comprehensive, and interdisciplinary team-based approach for people with PD and movement disorders and their care partners. Dr. Goldman is the fellowship director for the first-ever PD and Movement Disorders Neurorehabilitation fellowship at Shirley Ryan AbilityLab. Dr. Goldman is a core faculty member for the Allied Team Training for PD course offered by the Parkinson’s Foundation and serves on the Parkinson’s Foundation Scientific Advisory and Center of Excellence Boards. She is currently the Chair of American Academy of Neurology (AAN) Movement Disorders Section and the Lewy Body Dementia Association Scientific Advisory Committee, past chair of the international Movement Disorder Society (MDS) Pan-American Section Education Committee (2015-2019), and Secretary-Elect for the MDS Pan-American Section. She also serves on the MDS Study Groups for PD-MCI Validation and Neuroimaging, MDS and MDS-PAS Congress Planning Committees, and Task Forces on Leadership and Interdisciplinary and Integrated care; the AAN Women in Leadership Committee; and Editorial boards of the Movement Disorders journal and Journal of Clinical Movement Disorders and as an Associate Editor for Frontiers in Neuroscience Neurorehabilitation journal.