Looking back, Walter F. Mondale isn't sure when the first sign of his wife Joan's illness appeared. “I realized that she had started walking more slowly, and I didn't know what that meant. I wasn't really suspicious at first; I just thought she wanted to walk more slowly,” recalls the former US Vice President and 1988 presidential candidate, who also served two terms as a Minnesota senator.
But things started to get worse. “Her walking continued to get slower, and sometimes weaker, and I could see that her face—that beautiful face—was starting to freeze up,” Mondale says. “There was a long period where we weren't sure what it was. For maybe about two years, we had been going down to the Mayo Clinic in Rochester trying to figure out what was happening.”
Mondale will never forget the day in 2010 when he and Joan sat down with Ronald C. Petersen, MD, PhD, director of Mayo's Alzheimer's Disease Research Center and a Fellow of the American Academy of Neurology (FAAN), and J. Eric Ahlskog, MD, PhD, a professor of neurology at Mayo Medical School and former chair of the Mayo Clinic division of movement disorders, and learned exactly what was wrong: Joan, then in her late 70s, had Lewy body disease with parkinsonism and dementia. (To learn more about the disease, see “A Different Kind of Dementia” on page 21.)
“It was a hard meeting,” Mondale says slowly. “Joan asked whether there was anything that might get better, anything that could be done to minimize some of the hard stuff. And they told her no. They said, ‘We are unaware of anything that will help.’ As painful as that was, it helped us realize what we were dealing with. They were very nice about it, but they had that meeting partly because they wanted to be clear.”
FACING DEMENTIA HEAD ON
An artist, author, and avid patron of the arts who had earned the affectionate nickname “Joan of Art” and showcased great American artists such as Robert Rauschenberg, Edward Hopper, and Louise Nevelson at the Vice President's mansion during her husband's term in office, Joan Mondale was “very practical-minded” about her disease, her husband recalls. “She told me early on that she had listened again to the Mayo specialists and read the little pamphlet they put out, and she said, ‘This is an awful disease I have.’ She understood what was going on.”
With the help of visiting health aides and, ultimately, live-in assistance, Joan was able to remain at home even as her health declined. “We had really good people, and Joan liked them, and they took great care of her. Being able to do all that at home was the biggest plus, because this was her house, and I think it made a big difference in how she felt about herself.”
Because he had largely retired from public life and had returned to practicing law in Minnesota, Mondale could also care for his wife. “I could measure what I would do and when I would do it, and people understood,” he says. “We had a limited social life, but we did have a social life. Our family was around. We could go out for dinner once in a while—birthday parties, Christmas parties, that sort of thing. But then it really hit, and there wasn't much you could do at the end.”
On February 2, 2014, the family announced that Joan had entered hospice care; she died the following day at the age of 83.
A SECOND TRAGEDY
It was the second time in three years that Mondale had lost one of the people he loved most in the world to a neurologic illness. In 2011, his daughter, Eleanor Mondale Poling, had died of a brain tumor at age 51, after battling the disease for six years.
When Eleanor was originally diagnosed in 2005, “It was such a shock. She'd always been the healthiest person in our family,” says Mondale, who also has two sons, Theodore and William. Eleanor's original course of radiation and chemotherapy appeared to nearly eradicate the tumor, so when it returned in 2008, the Mondales remained optimistic.
“At first we had a good deal of hope. We took her to Mayo, and she had wonderful doctors there. We thought maybe we could beat it,” Mondale says. “But as the disease went along, it became more apparent that we were going to lose her. And one day, our doctor at Mayo said, ‘We may have to think about hospice care.’ We didn't care what it cost. We wanted her to have the best, but they couldn't do anything for her when it got down to the end.”
FIGHTING BRAIN DISEASE
Long active with the Mayo Clinic Foundation, Mondale's personal losses have left him more determined than ever to do what he can to fight neurologic illness. To that end, he has signed on as the honorary chairman of the Board of Directors of the American Brain Foundation in Minneapolis, MN (americanbrainfoundation.org), which raises funds for brain disease research.
“I'd like to know where the hopeful avenues are, and I'd like to go down those roads and help our doctors and research centers make more progress,” he says. “That's a pretty arrogant statement, but if I can help, I want to help.”
Mondale has long been interested in health care. As a senator from 1964 to 1976, he served on committees related to the National Institutes of Health and worked on passing legislation to expand Medicare and Medicaid in an effort to improve health care for all Americans.
He opposed multiple efforts by the Nixon administration to cut medical research funding, declaring, “It seems incredible to me…that a nation willing to spend billions of dollars on defense procurement and supersonic transports lacks the will to support desperately needed research on neurologic disease, cancer, stroke, diabetes, and so many other illnesses.”
“I know [our nation's leaders] have done some good, but we didn't go as far as we'd hoped,” says Mondale today. “The dreams we in Congress had that disease would give way just because you provided some funding didn't quite hack it.”
But Mondale remains hopeful that building on the progress that has been made thus far will ultimately lead to effective treatments, and he sees his role with the American Brain Foundation as one that will help generate important new research funding. “Somebody told me that if we could find a breakthrough on Alzheimer's, not only would we improve countless lives, but we'd save a trillion dollars a year.”
That's what makes the recent gift of $5.75 million from the Harry T. Mangurian Jr. Foundation in Palm Beach, FL, to the Mayo Clinic's campus in Jacksonville, FL, for research into Lewy body dementia so heartening. The new Mayo Clinic Dorothy and Harry T. Mangurian Jr. Lewy Body Dementia Program will be one of only a few in the world dedicated to the study of this disease.
It will build on research from scientists at the Mayo Clinic, who reported in 2013 that a rare sleep disorder known as rapid eye movement (REM) sleep behavior disorder, or RBD—in which people violently act out their dreams—may predict the development of Lewy body dementia as much as 10 to 20 years before other symptoms appear.
Also in that year, an international research team found that mutations in a gene known as GBA1 are associated with an increased risk of having Lewy body dementia, and of developing it at a younger age.
Still more research has determined that the APOE ∊4 gene, a known risk factor for developing Alzheimer's, also increases the risk of developing Lewy body disease. This indicates that the two conditions might share some root causes, which could mean that future therapies developed to treat one disease might be helpful in treating the other.
As Mondale has emphasized throughout his career, research, and the funding for it, “is absolutely essential.”
A Different Kind of Dementia
About 1.3 million Americans currently have Lewy body dementia, compared to about 5.5 million people who have Alzheimer's, according to estimates from the Lewy Body Dementia Association.
“It's the most common disease you've never heard of,” says James E. Galvin, MD, MPH, a professor of neurology at New York University Langone Medical Center, a member of the American Academy of Neurology, and an expert in the disease. Joan Mondale was not the only famous face of Lewy body disease; comedian Robin Williams and deejay Casey Kasem also had it.
Lewy bodies are abnormal clumps of a protein called alpha-synuclein that form inside the nerve cells in the brain that control memory and movement. The buildup of these clumps disrupts the activities of chemicals that are important to brain function and interferes with these nerve cells, eventually causing them to die.
Diagnosis Is Difficult
The disease is tricky, says Dr. Galvin. “It's not a classic 1-2-3 presentation of symptoms, which means people can have difficulty getting a diagnosis.” And there are no typical signs on an imaging scan; the MRI of a person with Lewy body dementia looks similar to that of a person with Alzheimer's disease.
Dr. Galvin's team surveyed nearly 1,000 pairs of patients who had Lewy body dementia and their caregivers and found that it was common for them to have seen many physicians multiple times over an 18-month period before getting a diagnosis. The disease can be confused early on with depression or an anxiety disorder, or, as symptoms progress, with another type of dementia or Parkinson's disease.
It's important to get an accurate diagnosis, since not all treatments for one disease are appropriate for the other. For example, the antipsychotic medications that are often used to help ease the distress and aggression of Alzheimer's may cause serious side effects like confusion, delusions, and impaired swallowing in people with Lewy body dementia.
Treatment Is for Symptoms Only
As Walter and Joan Mondale discovered, no specific treatments for the disease currently exist. “We have lots of treatments for individual symptoms, such as Parkinson's medications for movement symptoms, Alzheimer's medications for memory problems, and stimulants for attention problems, but we're not that far into finding disease-modifying drugs,” Dr. Galvin says. “We're far behind the Alzheimer's world.”
Scientists are just beginning to understand how alpha-synuclein—the basic building block of the Lewy bodies—develops to form the characteristic masses in the brain, which so far can be seen only on autopsy. “We're probably a full decade behind the research on amyloid [the sticky protein that accumulates in the brain] in Alzheimer's disease,” Dr. Galvin says.
People who have Lewy body dementia and their friends and family can find support, advice, and resources at the Lewy Body Dementia Association (lbda.org). To learn more about Lewy body dementia, visit the National Institute of Neurological Disorders and Stroke information page at http://bit.ly/NINDS-Lewy.
Is It Lewy Body or Alzheimer's Disease?
Early on, Lewy body dementia is often confused with either Alzheimer's disease, if the person has more obvious cognitive problems, or Parkinson's disease, if the earliest signs point to a movement disorder. Indeed, people who have Lewy body dementia may also have either Alzheimer's or Parkinson's disease.
“More than two-thirds of patients with this disorder also have Alzheimer's disease,” says John C. Morris, MD, the Friedman Distinguished Professor of Neurology and director of the Knight Alzheimer Disease Research Center at Washington University School of Medicine in St. Louis, a Fellow of the American Academy of Neurology, and a member of the Neurology Now editorial advisory board. “That's why it's not uncommon for patients to be diagnosed with Alzheimer's first, as they express the signs and symptoms of that disease as well.”
But Lewy body dementia has unique characteristics. Here are some of the ways it differs from Alzheimer's:
Patients tend to be younger. “The average patient with Lewy body dementia is in his or her early to mid-70s, while the average Alzheimer's patient is in his or her late 70s to early 80s,” says James Galvin, MD, MPH, a professor of neurology at New York University Langone Medical Center and an expert in the disease. The average time of survival after diagnosis, though, is the same for both diseases: about eight years.
Memory may be more intact. “In Lewy body dementia, memory is better preserved, relatively speaking,” says Dr. Galvin. “People have more problems with staying focused and planning and carrying out tasks.”
Vision is affected. Many patients initially think they have a problem with their eyes and visit the eye doctor several times trying to find the right prescription. Researchers suspect this is because the disease disrupts certain visual pathways in the brain.
There are more motor symptoms. Patients often experience rigidity or stiffness, balance problems, difficulty initiating movements, and foot shuffling.
Hallucinations start earlier and are sharper. “Fairly early on in the disease, people may see things that aren't there or visually misperceive objects,” says Dr. Galvin. The hallucinations are well-formed and frequently take the shape of a childish or elfish person or a fuzzy animal, and are not frightening, he says.
Alertness, attention, and concentration can change spontaneously. People who have Lewy body dementia may come across as sleepy or lethargic, spending a lot of time with their eyes closed or with a blank stare. Their train of thought may come across as illogical and incoherent. But at other times, they may be quite lucid and able to function well.
Sleep disturbances are more common. Many patients act out their dreams, says Dr. Galvin, meaning they may thrash, punch, kick, or shout during sleep.
More symptoms affect involuntary processes. Patients sometimes gradually lose their sense of smell or have constipation or urinary problems. Some experience orthostatic hypotension, a condition in which blood pressure drops upon standing and patients feel faint.
Forging a Healthcare Legacy
1966: Elected to the Senate for the first time.
2015: Received the ABF's Public Leadership in Neurology Award for his dedication to advancing public understanding and awareness of neurologic disease, advocating for research, and contributing significantly to improve patient care.
© 2015 American Academy of Neurology
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