The second most common form of dementia is virtually unknown to most people. However, Lewy body dementia affects 1.4 million Americans, with symptoms commonly misdiagnosed as Alzheimer’s disease. Additional symptoms such as hallucinations and uncontrollable shaking make diagnosis and caregiving more difficult, and treatments for Alzheimer’s or psychosis can often be harmful. Experts discuss.
- Candy Schulman, daughter of woman who died with Lewy body dementia
- Dr. James Leverenz, Director, Cleveland Clinic Lou Ruvo Center for Brain Health and Chair, Scientific Advisory Council, Lewy Body Dementia Association
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16-28 Lewy Body Dementia
Reed Pence: Candy Schulman’s mother, Sylvia Wechsler, was in her late 80’s when she started having trouble with a task she did every year—organizing a big holiday dinner.
Candy Schulman: She couldn’t get the guest list right and she would forget names and she would cross them out and she wanted to get a count, was there going to be 20, 21, 22. And she kept asking me to help her with the guest list. But she wasn’t used to asking for help so she got very frustrated and a little teary. And then what happened was she would rip up the paper and start again.
Pence: That was unusual for her mother, but Schulman, a professor of writing at the new school in New York, figured it was simply part of natural aging. She wasn’t too worried. After all, her mother was still driving and even golfing regularly. But then other things started going wrong.
Schulman: She did start hallucinating, she had these sculptures in her living room and she thought the sculptures were moving and doing bizarre things. And she would call me over and say “Don’t you see that happening?” And I didn’t. She also had hallucinations where she thought people were trying to kill her and rob her and she would call the police, she would call 911.
Pence: Doctors had little to say about Sylvia’s condition. And what they did say wasn’t very helpful.
Schulman: She was misdiagnosed, you know, in the beginning it was “Well what do you expect at your age?” doctors would say to her. Not the doctors that we returned to necessarily. And then some doctors would say it was a normal aging process. And most doctors were befuddled, she was misdiagnosed with illnesses. No one ever diagnosed her with Alzheimer’s because she didn’t have an official diagnosis until about a year or two before she died. She was put on a lot of medications that can be harmful.
Pence: Schulman figures that for about eight of the 10 years before her mother’s death, she was misdiagnosed. She clearly had dementia but with hallucinations, and jerky involuntary body movements that are not characteristic of Alzheimer’s.
Schulman: One of the things that started happening was she started losing her balance before the jerky movements. And she would fall and she would lose her balance and actually in the last two years of her life, she did get up and fall and break her hip and she was never able to walk again. And the balance issue looks a little like Parkinson’s and that’s why it’s so hard to diagnose. So towards the end of her life, she would have these jerky motions with her hands and sometimes we’d have to put mittens on her hands so she wouldn’t scratch herself. That’s the part I didn’t know how to help her with, the balance, the jerky movements that were so upsetting. So she was being treated for the hallucinations as though she were schizophrenic which she wasn’t, there isn’t a lot you can do. Mainly you need to keep our loved ones safe from falling but you can’t always watch someone.
Pence: Schulman’s done a lot of research into those symptoms in conjunction with the book she’s writing, titled Raising My Mother. Since Sylvia’s death in 2009, Schulman has concluded that her mother suffered from the second most common form of dementia, suffered by about 1.4 million Americans, or 20 percent of people with dementia. It’s called Lewy body dementia, spelled L-e-w-y after the pathologist who discovered the triggering changes in the brain. However, Lewy body dementia is really an umbrella term for two disorders.
Leverenz: One is actually Parkinson’s disease dementia, so dementia that occurs in the context of somebody who already has classic Parkinson’s disease with the motor symptoms that you typically see with that such as the tremor, the slowness of movement, stiffness of muscles.
Pence: That’s Dr. James Leverenz, director of the Cleveland Clinic Lou Ruvo Center for Brain Health and Chair of the Scientific Advisory Council for the Lewy Body Dementia Association.
Leverenz: The other disorder underneath that umbrella is dementia with Lewy body and these individuals, unlike the Parkinson’s disease, the dementia patients present with a cognitive or thinking problem. They may later develop motor symptoms of Parkinson’s or some of the other non-motor complications of Parkinson’s, but the big difference is they present with a cognitive impairment verses a motor impairment.
Pence: Comedian Robin Williams was known to be depressed before his suicide in 2014. But since then, blame has been centered on Lewy body dementia. Williams’ widow says he was being treated for early Parkinson’s disease and an autopsy confirmed the presence of Lewy bodies in his brain.
Leverenz: Lewy bodies are a change we see in the brain, it’s actually in the brain cells. It’s an aggregation of protein that’s call alpha-synuclein and we see Lewy bodies both in Parkinson’s disease, Parkinson’s disease with dementia and dementia with Lewy bodies. That’s why we kind of put them under that umbrella term of Lewy body dementia. And Alzheimer’s disease, there are different proteins that aggregate in the brain. Amyloid and something called PAU, P-A-U, so the protein that aggregates in Lewy bodies is a different protein but it’s very similar in that way. It tends to occur in different parts of the brain, say in Alzheimer’s verses the Lewy body disorders, which explains why we see different kinds of symptoms in these disorders.
Pence: However, those differences are often overlooked or may seem to doctors to be completely unconnected to what’s mainly a cognitive problem. Leverenz says as a result, Lewy body dementia is often misdiagnosed. Reportedly, the average patient spends a year and a half looking for a diagnosis visiting at least three doctors along the way.
Leverenz: We see dementia with Lewy bodies often diagnosed as Alzheimer’s disease and that’s partly, no fault necessarily of the doctors who are trying to make that diagnosis. Sometimes it just looks like a pure thinking problem, cognitive impairment problem. And therefore, they don’t see some of these characteristics such as hallucinations, seeing things that aren’t real. Something called REM sleep disorder where people act their dreams out at night. We don’t see it in Alzheimer’s unless they have coexistence so have both Alzheimer’s and Lewy body changes in the brain. Little bit easier when it occurs in the context of Parkinson’s disease because these people generally clearly already have a motor disorder and they’re responding to therapy. We know they have Parkinson’s disease, and in that context, about 80 percent of patients, if they live long enough, will develop dementia.
Pence: Leverenz says the cognitive deficits of Lewy body dementia may not be as severe as in Alzheimer’s but the additional symptoms can be extremely serious. Hallucinations are extremely common. They occur in about 70 percent of patients.
Leverenz: They’re often very vivid, they’re visual, people will see children playing in their front yard or animals in the house. And they can be very vivid and very real looking. They are not always disturbing and because the medications for treating them can have significant side effects, if they’re not disturbing, we often don’t treat them. But if we have to, we use the medications that were originally developed for disorders like schizophrenia to try to suppress the particularly, the frequency but also the emotional reaction to them.
Pence: When Lewy body dementia is misdiagnosed as Alzheimer’s and its hallucinations are treated as a completely separate case of psychosis or schizophrenia, doctors may treat with antipsychotics too quickly. It may be exactly the wrong treatment.
Leverenz: The antipsychotics which we use very judicially in this patient population can really aggravate the motor symptoms of Parkinson’s disease that you see both in Parkinson’s dementia as well as dementia with Lewy body. So people can be exquisitely sensitive to even low doses and have fairly severe reactions in terms of becoming very rigid. Some have to be hospitalized and have IV’s or feeding tubes placed because they are so rigid and they can’t really move. So we have to be very careful. Some of the medications are a little better tolerated than the others, but if we can avoid using them, we do. When we have to use them, we start at very low doses and we’re very careful to pick which ones we want to use.
Pence: Yet another of the unique symptoms, REM sleep disorder, can also be disturbing.
Leverenz: Typically what I’ll ask a family member is, “Do they act their dreams out at night?” That’s the first clue to what’s going on and what happens for most of us during dream sleep is that we’re essentially paralyzed. We don’t really move much; we may talk a little bit in our sleep. Generally we’re not moving around much because of this sort of normal physiologic paralysis that happens during dream sleep. For some reason, in the Lewy body dementias, that paralysis, that lack of movement is released and people start acting their dreams out. Often the spouse will say, “Well actually I had to move to a different room because they were hitting me at night or waking me up. They were moving so much.” Occasionally, people will even fall out of bed, things like that.
Pence: Leverenz says those movements may be extremely violent. Once awakened, people often say they’re being chased, or were chasing someone else. Medications may help, but if melatonin doesn’t work, doctors may need to prescribe muscle relaxants, and those can promote drowsiness. For some patients, that’s the last thing they need. Daytime sleepiness is yet another unique symptom of Lewy body dementia.
Leverenz: Saw a patient yesterday with dementia with Lewy bodies. He sleeps about 17 hours a day, so he’ll sleep a good 12 hours at night, get up to have breakfast and then go right back to bed and sleep some more so we can see this, what we call hypersomnia, where people sleep many hours.
Pence: Leverenz says some medications that were originally developed for Alzheimer’s disease have shown some positive effects for symptoms of Lewy body dementia. But like Alzheimer’s, there’s no real treatment for the disease itself. Caregiving can also be a lot like with Alzheimer’s… only worse.
Leverenz: There are some similarities in that you’re talking about a change in a person’s cognitive capacity to manage day-to-day actives so the person who used to take care of the finances or could be trusted to take a message or those sorts of things will change and suddenly the caregivers find themselves doing all of those things. What’s a bit different in the Lewy body dementias is they have more of the psychiatric symptoms we mentioned, the hallucinations, they have motor symptoms, they can’t move as well. They can look like they have Parkinson’s because some of them do. There are sort of multiple parts of what make you functional, not just the thinking skills, but the motor skills and even some of the behavioral changes can make it particularly difficult for caregivers.
Pence: Leverenz says scientists hope more funding for research on this, the second most common form of dementia. You can find out much more information on Lewy body dementias at the association website, LBDA.org or through links on our website, radiohealthjournal.net. You’ll also find archives of our programs there, as well as on iTunes and Stitcher.
I’m Reed Pence.