A caregiver in our local support group follows news about Dennis Dickson, MD, at Mayo Clinic, as he's the neuropathologist who studied her husband's brain upon donation to Mayo Jax. She sent me this wonderful Mayo Clinic article on Lewy body dementia and research going on at Mayo. It refers to the $1 million donation give to Mayo to study LBD.http://www.mayoclinic.org/mcitems/mc230 ... 6-2502.pdf
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The Complexity of Discovery
Mayo Clinic Magazine
2011 Issue 2
An hour or even a day isnât enough time to talk to Gordon Latz and Steve Mehallis of Ft. Lauderdale, Fla.
Former business associates of Harry T. Mangurian, Jr., Latz and Mehallis now direct Harry Mangurianâs foundation, but they can entertain for hours with stories about the old days with Harry, who passed away in 2008, and his wife, Dorothy (Dottie).
âWorking for Harry was a revelation,â says Mehallis, who left an executive position at a large accounting firm to become the financial adviser for many of Mangurianâs business pursuits, including the Boston Celtics. Mangurian purchased the Celtics in 1979, just as the team was about to draft Larry Bird and to assemble one of the best rosters in the history of the NBA.
âThe pecking order at big companies is incredible. But with Harry it was like you were family and he let you do your job. At the end of the day, though, he was the decision maker and he did have the Midas touch. The acquisition and development of the Celtics was a perfect example. His timing was always impeccable.â
Fortune favored him in his personal life, too, says Latz, who was Mangurianâs operations manager for nearly four decades. âHarry Mangurianâs wife, Dottie, made you feel like she was your best friend as soon as you met her. Everyone fell in love with her right away.â
Thereâs more than a hint of wistfulness when Latz and Mehallis talk about Dottie though. At the mention of her name, their eyes drop to the floor for a moment. Their words are a little measured, and a somber feeling enters the room.
Although Harry Mangurian passed away three years ago, at age 82, from leukemia, Dottie is still alive. Yet the Dottie that everyone remembers started disappearing more than 10 years ago due to a mysterious brain disorder that began subtly.
âI used to walk on the beach every weekend with Dottie,â Latz recalls. âGradually, the distance decreased. Then she couldnât walk anymore and the hallucinations started, so weâd go out on the patio to enjoy the sun. Now she sleeps about 22 hours a day and barely says a word. At first, the doctors told us she had Alzheimerâs disease and Parkinsonâs disease. But then we learned it was something else; something called Lewy body dementia.â
âLike Alzheimerâs and Parkinsonâs, only worseâ
Lewy body dementia in many ways resembles a combination of Alzheimerâs disease and Parkinsonâs disease. It often impairs movement and memory. Plaques and other brain pathology consistent with Alzheimerâs and Parkinsonâs are found at autopsy in patients with this disease. There are no treatments to slow the progress of Lewy body dementia: only symptomatic therapies that improve quality of life.
But it would be a mistake to say the disease is a combination of two other disorders, says Dennis Dickson, M.D., a neuropathologist at Mayo Clinic in Florida, who has studied Lewy body dementia for two decades. âWe talk to family members of patients, and they say âItâs like Parkinsonâs and Alzheimerâs, only worse,ââ Dickson says. âThe combination of disease processes seems to be more than additive; itâs synergistic. So patients seem to have greater impairment than we would expect given the amount of pathology we see at autopsy.â
Thatâs just one of the unpleasant surprises that go along with Lewy body dementia. Its constellation of symptoms confounds many physicians, who often mistake it for Parkinsonâs, Alzheimerâs or even schizophrenia because patients often have hallucinations. At the same time, patients are especially sensitive to some of the treatments for those disorders, particularly anti-schizophrenia drugs, and the wrong treatment can exacerbate symptoms or even be deadly.
The disease isnât a rarity in the dementia world either. More than 1 million Americans have the disease, and itâs common, at autopsy, to find some degree of Lewy body dementia pathology in patients who are diagnosed with Alzheimerâs disease. âIn some Alzheimerâs disease populations, as many as 60 percent will have some degree of Lewy body dementia pathology,â Dickson says. âSo the picture of all dementias is becoming more mixed. But, we are now realizing that Lewy body dementia is more common than we once thought.â
A sleeping giant
Given all the complexities and challenges of understanding Lewy body dementia, perhaps itâs surprising that researchers at Mayo Clinic hold such hope for improving the future treatment of the disease. And sleep â perhaps the most prominent aspect of Dottie Mangurianâs existence today â is one of the main reasons.
Mayo Clinic researchers are finding evidence suggesting that excessive daytime sleepiness is a potential indicator of early-stage Lewy body dementia. But their sleep research has gone even further, finding a link between an uncommon sleep disorder, called REM sleep behavior disorder (RBD) and Lewy body dementia.
The finding comes from two decades of research led by Bradley Boeve, M.D., a neurologist; Tanis Ferman, Ph.D., a neuropsychologist; Dr. Dickson and several other Mayo Clinic colleagues. Patients with RBD act out their dreams, often violently, and the Mayo team has discovered that patients with RBD have an increased risk of developing Lewy body dementia.
That discovery offers a potential road to early diagnosis, improved quality of life and, eventually, treatments that finally hit the diseaseâs root causes.
Why? Because RBD can occur years â even decades â before the first signs of Lewy body dementia.
âEarly diagnosis is incredibly important,â says Boeve. âQuality-of-life treatments have the most benefit early on, and weâve found we can make a tremendous difference if we can start treatment earlier. Plus, a comprehensive approach toward management, including treatment of all sleep disorders, provides the best opportunity for clinical improvement.â
Looking further down the road, Boeve says the RBD discovery and other possibilities for early diagnosis will help make treatments more effective. Earlier diagnosis means earlier treatment. âOnce we have therapies that actually modify the disease, not just the symptoms, it makes sense that the impact of those therapies will be greatest when started as early as possible.â
Although the RBD connection is a fruitful avenue for ongoing research and early diagnosis, the Mayo Clinic researchers all agree that itâs just one lead in the mystery that is Lewy body dementia. The solution will come when the various leads from across the spectrum of research come together. âWe need to establish a definitive âprofileâ for Lewy body dementia,â Ferman says. âOne that fully defines the cognitive, clinical and pathological features of the disease. With that, weâll make early, accurate diagnosis possible and help identify new, more effective therapies.â
The Mangurian Foundation legacy at Mayo Clinic
Through their leadership of the Harry T. Mangurian, Jr., Foundation, Latz and Mehallis are helping Mayo Clinic identify Lewy body dementiaâs signature, and generate new possibilities for treating the disease. The foundation recently made a gift to support research on multiple fronts.
In addition to genetics, pathology and cognitive studies, the foundationâs support is helping Kejal Kantarci, M.D., a rising star in the radiology field, to develop a comprehensive imaging test. Kantarci is combining three types of imaging techniques, each of which provides information on different Lewy body dementia pathologies, to help diagnose the disease and differentiate it from Alzheimerâs.
Another recipient of the foundationâs funds, ShuHui Yen, Ph.D., is refining a cellular model of the disease. Itâs one of the only models of its kind in all medical research. Not only is this model useful for understanding the formation of Lewy bodies, which are twisted bits of a protein called alpha synuclein, itâs also one of the first tools scientists will use to identify new therapies for the disease.
The breadth of research highlights an important and even a unique aspect of Mayo Clinicâs Lewy body dementia research program. âSome places have good pathology programs for Lewy body dementia and others have good clinical research,â Dickson says. âMayo has both and we have good genetics research, too. But we arenât stopping with what weâve got. Weâre recruiting more young scientists as well.â
Harry Mangurian would have applauded that news. âHe was always looking ahead, and he was a marketer at heart,â Mehallis says. âHe believed we could beat Lewy body dementia if we could get more people involved and if we made more people aware of the disease.â