Bill Buckner, the famed Boston Red Sox hitter who also famously missed a ground ball during the 1986 World Series, died Monday after a battle with dementia with Lewy bodies (DLB), a brain disease that is far less known than Alzheimer’s disease. Comedian Robin Williams also had it and CNN founder Ted Turner announced last September that he had it.

Despite its relatively low profile, Lewy body dementia is responsible for 5 percent to 10 percent of dementia cases, making it the third most common cause of dementia, according to the Alzheimer’s Association. Alzheimer’s and vascular dementia head the list. Patients often have more than one type of dementia, which makes diagnosis challenging.

Lewy body dementia is characterized by the accumulation of Lewy bodies in the brain. These are deposits of the protein alpha-synuclein, which also cause Parkinson’s disease. When patients with Parkinson’s are followed for many years, about 80 percent develop cognitive impairment, said David Irwin, an expert on DLB at Penn Medicine. And, most people initially diagnosed with DLB eventually develop Parkinsonism. DLB and Parkinson’s disease dementia — the term for dementia that starts a year or more after physical symptoms — are indistinguishable, but people who experience cognitive symptoms first tend to fare worse.

David Irwin is a cognitive neurologist at the Penn Frontotemporal Degeneration Center.
Courtesy of Penn Medicine
David Irwin is a cognitive neurologist at the Penn Frontotemporal Degeneration Center.

In Alzheimer’s disease, different malformed proteins build up in the brain. It is common for those with dementia to have both the plaques and tangles of Alzheimer’s and Lewy bodies.

Was it unusual for Buckner to die of DLB at age 69?

Not really, Irwin said. While doctors typically expect to see DLB symptoms in people over age 65, it can develop earlier. On average, people live seven years with the disease but there are cases where it progresses very rapidly.

How is it different than Alzheimer’s disease?

There is considerable overlap in symptoms, particularly as the diseases progress. The first symptom of Alzheimer’s disease is often poor short-term memory. People with DLB, Irwin said, are more likely to have these symptoms first:

  • Well-formed visual hallucinations including people and animals. People with DLB often know these aren’t real while people with Alzheimer’s are more prone to false beliefs.
  • Periods of inattention or disorganized speech. People with DLB sometimes visit emergency departments because of suspected stroke, but their functioning slowly returns to baseline.
  • Difficulty with executive functioning, attention and spatial skills.
  • Cognitive changes paired with physical problems, including impaired balance and tremor when reaching for something. Changes in autonomic functions can lead to poor blood pressure control (feeling faint while standing), constipation or bladder control.

Those with REM sleep behavior disorder, in which people violently act out their dreams, are at high risk for DLB.

Is there a treatment?

There’s no cure, Irwin said, but there are medicines that improve physical and cognitive symptoms.