The recent death of former Red Sox first baseman Bill Buckner has once again raised the public profile of Lewy body dementia (LBD), a common but still frequently misdiagnosed form of neurodegenerative disease.

LBD, which affects more than 1.3 million people in the United States, surged into the public spotlight in 2016 when Susan Schneider Williams, the wife of the late Robin Williams, revealed that the actor and comedian had been diagnosed with Lewy body dementia in the months prior to committing suicide in 2014.

What is Lewy Body Dementia?

Stephen Gomperts, MD, PhD, a physician-investigator in the Department of Neurology at Massachusetts General Hospital, explains that LBD is actually an umbrella term for two different diseases—one is dementia with Lewy bodies (LBD) and the other is Parkinson’s disease with dementia, which afflicts about 30% of people with Parkinson’s disease (PD).

LBD is the second most common cause of progressive dementia after Alzheimer’s disease.

Clinically, patients with LBD develop progressive trouble thinking that interferes with their activities of daily living, as well as progressive motor impairments including motor slowing, tremor, rigidity and walking changes.

In dementia with Lewy bodies and often in PD dementia, patients can develop visual hallucinations, fluctuations in levels of alertness and thinking and a condition called REM sleep behavioral disorder, in which they lose the normal paralysis of REM sleep and start acting out their dreams.

Patients with dementia with Lewy bodies develop trouble thinking right away and motor problems may arise at that time or later, Gomperts explains. Patients with Parkinson’s disease dementia experience these motor changes up front and develop trouble thinking later as the disease progresses.

The cognitive challenges experienced by LBD patients often differ from the more commonly known symptoms of Alzheimer’s disease. In Alzheimer’s, patients develop an early problem with short term memory, and over time other thinking problems start to accrue.

In LBD, patients may also develop early short term memory loss, but more often develop early and significant problems with planning and problem solving or with visual spatial function.

Treatment Options

Gomperts, who is Director of the Lewy Body Dementia Unit at Mass General and also sees patients in the Memory Disorders Unit and the Movement Disorders Unit, notes that the hospital’s clinical resources can ease the symptoms of LBD patients and improve the quality of life of patients and their caregivers.

“We have effective treatment strategies that can help with impairments of thinking, movement, hallucinations and sleep. We’re well positioned to help.”

The Lewy Body Dementia Association has also designated Mass General as a Research Center of Excellence.

Research Efforts

Gomperts and his group are using position emission tomography (PET) molecular imaging to learn more about the changes that occur in the brains of LBD patients.

While the signature brain change of LBD patients is the accumulation of Lewy bodies, changes associated with Alzheimer’s disease are frequently present as well, including the accumulation of amyloid plaques and tau tangles.

“We suspect that Alzheimer’s changes, when present, are not innocent bystanders but contribute to the disease process in LBD patients. We’re working to understand this. We expect that breakthroughs in the treatment of Alzheimer’s will help patients with LBD as well,” Gomperts says.

Gomperts is also collaborating with Changning Wang, PhD of the Martinos Center for Biological Imaging to investigate the role in LBD of key proteins called histone deacetylases (HDACs) that influence the expression of genes in the brain.

They are currently looking at HDAC expression in LBD patients with PET imaging to determine how it contributes to LBD and whether it could be useful as a clinical tool or treatment target.

To Gomperts, it makes sense to use the shared disease features of dementia with Lewy bodies and Parkinson’s disease to accelerate understanding and treatments.

“Because the brain changes of Parkinson’s and dementia with Lewy bodies are virtually identical, the insights that we develop into one of these diseases should inform our understanding of both.”

How You Can Help

Those interested in helping with research in the Gomperts Lab can do so by enrolling as a research participant or by providing philanthropic support, which helps the lab to investigate promising new hypotheses that may be too early for more traditional forms of research funding. To learn more, please contact the Gomperts Lab.

Additional Resources:

• MGH Memory Disorders Division
• Lewy Body Dementia Association
• What is Lewy Body Dementia? (National Institute on Aging)
• Top Resources for Lewy Body Dementia (Brain Support Network)

About the Mass General Research Institute
Research at Massachusetts General Hospital is interwoven through more than 30 different departments, centers and institutes. Our research includes fundamental, lab-based science; clinical trials to test new drugs, devices and diagnostic tools; and community and population-based research to improve health outcomes across populations and eliminate disparities in care.
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