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Baseball Great Dies of Lewy Body Dementia

— So, what is the disease that took Bill Buckner's life?

MedpageToday
An old photo of Boston Red Sox first baseman Bill Buckner.

Former Boston Red Sox first baseman Bill Buckner has died at the age of 69. The cause of death was Lewy body dementia. Buckner's wife, Jody, confirmed his death saying: "After battling the disease of Lewy Body Dementia, Bill Buckner passed away early the morning of May 27th surrounded by his family. Bill fought with courage and grit as he did all things in life. Our hearts are broken but we are at peace knowing he is in the arms of his Lord and Savior Jesus Christ."

Although Buckner was an accomplished player, having played 22 seasons in the majors, winning a batting title in 1980 and being an All-Star, Buckner's legacy was darkened by a single error made in his career -- a ball passed through his legs allowing the New York Mets to win Game 6 of the 1986 World Series. The Mets went on to win Game 7 as well, denying the Red Sox a chance to break the 1918 World Series "Curse of the Bambino."

Although his teammates didn't blame Buckner for the loss, fans were not as generous. "When that ball went through Bill Buckner's legs, hundreds of thousands of people did not just view that as an error, they viewed that as something he had done to them personally," longtime Boston Globe columnist .

After the Red Sox won their second World Series in 2008, Buckner was welcomed back to throw the ceremonial first pitch. He received a two-minute long standing ovation from fans in the stands.

What is Lewy Body Dementia?

Dementia with Lewy bodies (LBD) is one of the most common types of progressive dementia.

According to the , LBD is not a rare disease. It affects an estimated 1.4 million individuals and their families in the U.S. Lewy body dementia typically begins at age 50 or older, although sometimes younger people have it. LBD appears to affect slightly more men than women. Average survival after the time of diagnosis is similar to that in Alzheimer's disease, about 8 years, with progressively increasing disability.

There are now several ways the LBD diagnosis can be made – on the presence of symptoms alone, or on the presence of fewer clinical symptoms plus a biomarker test result that suggests the presence of Lewy bodies in the brain. The new criteria were established at the 2015 International Dementia with Lewy Bodies Conference. A 2017 article in the journal Neurology .

Diagnosing LBD on Clinical Symptoms Alone

Dementia is required, which simply means a decline in thinking skills that interferes with everyday life. In early LBD, memory may be relatively normal in comparison to Alzheimer's disease. Instead, a person with LBD experiences problems with other cognitive skills, which may need a neuropsychologist for assessment:

  • Paying attention
  • Reasoning and problem-solving, called executive function
  • Understanding how objects relate in three-dimensional space, called visuospatial skills.

At least two of the following clinical symptoms are required:

  • Delirium-like fluctuating cognition: unpredictable changes in thinking, attention, and alertness
  • Repeated visual hallucinations
  • REM sleep behavior disorder (which may appear long before the dementia)
  • Parkinsonism, specifically slowed movements, tremor when limbs are at rest, and muscle rigidity

LBD also has other symptoms that support a diagnosis but are not so common that they help make the diagnosis. Two new supportive symptoms added to this list are the loss of smell and excessive daytime sleepiness.

Diagnosing LBD on Clinical Symptoms Plus Biomarkers

Criteria include:

1. Dementia plus one of the core clinical symptoms (fluctuating cognition hallucinations, REM sleep behavior disorder, parkinsonism)

2. At least one of the following biomarker test results

  • Brain scans (SPECT or PET) indicates a reduction in brain cells that produce dopamine
  • MIBG myocardial scintigraphy reveals reduced communication of the cardiac nerves
  • A formal sleep study confirms the presence of REM sleep behavior disorder, which in turn is defined by the Dementia with Lewy Bodies Consortium as "a parasomnia manifested by recurrent dream enactment behavior that includes movements mimicking dream content and associated with an absence of normal REM sleep atonia."

The Pathology of Lewy Body Dementia

Lewy bodies are named for Friedrich Lewy, a German neurologist. In 1912, he discovered abnormal protein deposits that disrupt the brain's normal functioning in people with Parkinson's disease. These abnormal deposits are now called "Lewy bodies."

Lewy bodies are made of a protein called alpha-synuclein. In the healthy brain, alpha-synuclein plays several important roles in neurons in the brain, especially at synapses. In LBD, alpha-synuclein forms into clumps inside neurons, starting in areas of the brain that control aspects of memory and movement. This process causes neurons to work less effectively and, eventually, to die. The activities of certain brain chemicals are also affected. The result is widespread damage to specific brain regions and a decline in abilities affected by those brain regions.

Lewy bodies affect several different brain regions in LBD:

  • The cerebral cortex, which controls many functions, including information processing, perception, thought, and language
  • The limbic cortex, which plays a major role in emotions and behavior
  • The hippocampus, which is essential to forming new memories
  • The midbrain and basal ganglia, which are involved in movement
  • The brain stem, which is important in regulating sleep and maintaining alertness
  • Brain regions important in recognizing smells (olfactory pathways)

Treatment of Lewy Body Dementia

While LBD currently cannot be prevented or cured, some symptoms may respond to treatment for a variable period of time. A comprehensive treatment plan may involve medications, physical and other types of therapy, and counseling. Changes to make the home safer, equipment to make everyday tasks easier, and social support are also very important.

Medications

Several drugs and other treatments are available to treat LBD symptoms.

Cognitive Symptoms

Some medications used to treat Alzheimer's disease also may be used to treat the cognitive symptoms of LBD. Cholinesterase inhibitors act on a chemical in the brain that is important for memory and thinking. They may also improve behavioral symptoms. The FDA has approved one Alzheimer's drug, rivastigmine (Exelon), to treat cognitive symptoms in Parkinson's disease dementia. This and other Alzheimer's drugs can have side effects such as nausea and diarrhea.

Movement Symptoms

LBD-related movement symptoms may be treated with a Parkinson's medication called carbidopa-levodopa (Sinemet, Parcopa, Stalevo). This drug can help improve functioning by making it easier to walk, get out of bed, and move around. However, it cannot stop or reverse the progress of the disease.

Side effects of this medication can include hallucinations and other psychiatric or behavioral problems. Because of this risk, physicians may recommend not treating mild movement symptoms with medication.

A surgical procedure called deep brain stimulation, which can be very effective in treating the movement symptoms of Parkinson's disease, is not recommended for people with LBD because it can result in greater cognitive impairment.

Sleep Disorders

Sleep problems may increase confusion and behavioral problems in people with LBD and add to a caregiver's burden. A sleep study may identify any underlying sleep disorders such as sleep apnea, restless leg syndrome, and REM sleep behavior disorder.

REM sleep behavior disorder, a common LBD symptom, involves acting out one's dreams, leading to lost sleep and even injuries to sleep partners. Clonazepam (Klonopin), a drug used to control seizures and relieve panic attacks, is often effective for the disorder at very low dosages. However, it can have side effects such as dizziness, unsteadiness, and problems with thinking. Melatonin, a naturally occurring hormone used to treat insomnia, may also offer some benefit when taken alone or with clonazepam.

Excessive daytime sleepiness is also common in LBD. If it is severe, a sleep specialist may prescribe a stimulant to help the person stay awake during the day.

Behavioral and Mood Problems

Behavioral and mood problems in people with LBD can arise from hallucinations or delusions. They may also be a result of pain, illness, stress or anxiety, and the inability to express frustration, fear, or feeling overwhelmed. The person may resist care or lash out verbally or physically. Caregivers must try to be patient and use a variety of strategies to handle such challenging behaviors. Some behavioral problems can be managed by making changes in the person's environment and/or treating medical conditions. Other problems may require medication.

Certain medications used to treat LBD symptoms or other diseases may also cause behavioral problems. For example, some sleep aids, pain medications, bladder control medications, and drugs used to treat LBD-related movement symptoms can cause confusion, agitation, hallucinations, and delusions. Similarly, some anti-anxiety medicines can actually increase anxiety in people with LBD.

Cholinesterase inhibitors may reduce hallucinations and other psychiatric symptoms of LBD. These medications may have side effects, such as nausea, and are not always effective. However, they can be a good first choice to treat behavioral symptoms. Cholinesterase inhibitors do not affect behavior immediately, so they should be considered part of a long-term strategy.

Antidepressants can be used to treat depression and anxiety, which are common in LBD. Two types of antidepressants, called selective serotonin reuptake inhibitors and serotonin and norepinephrine reuptake inhibitors, are often well tolerated by people with LBD.

Clinical trials working on the diagnosis and treatment of LBD can be found at .

Michele R. Berman, MD, and Mark S. Boguski, MD, PhD, are a wife and husband team of physicians who have trained and taught at some of the top medical schools in the country including Harvard, Johns Hopkins, and Washington University in St. Louis. Their mission is both a journalistic and educational one: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.