On Thursday the world learned through an Instagram post that Patrick Stewart had died the previous day “following a brief and poignant journey with Alzheimer’s Disease and Lewy Body Dementia.”
Just a few months earlier, the award-winning British actor known for his role as Captain Jean-Luc Picard in “Star Trek: The Next Generation” and his work in Shakespearean theater said in an interview with TV Insider: “I can’t imagine ever officially ‘retiring.’ I’ll always and forever be an actor.”
His death took many by surprise, as people often think of Lewy body dementia and Alzheimer’s as long, drawn-out conditions. That’s not always the case, medical experts say, and sometimes, the symptoms don’t make themselves known until the disease has advanced considerably.
“It’s hard to tell when a disease actually begins,” said Sami J. Barmada, associate director of the Michigan Alzheimer’s Disease Center and a professor of neurology at the University of Michigan Medical School. Some people have what he calls resilience — the ability to function relatively well despite changes in the brain.
“This means that the disease can be going on for a very long time, and they just don’t show many signs of that disease until it gets to a certain point, and then it moves very quickly. ”
Once someone develops Lewy body dementia, progression can range from approximately one to 10 years or longer, Barmada said. In some cases, however, dementia can progress over weeks and months, rather than years.
Rapidly progressive dementia, which can be any type of dementia, is generally defined as occurring over less than two years.
“Overall, rapidly progressive dementia is rare, affecting less than 5 percent of all people with dementia,” Barmada said, adding that 10 to 20 percent of rapidly progressive dementia is attributed to Lewy body dementia.
Signs of Lewy body dementia
Lewy body dementia is a debilitating disease thought to afflict more than 1 million Americans. Robin Williams grappled with the condition, and according to his widow, the actor and comedian killed himself because of the disease.
While in its early stages the condition may not interfere with everyday life, initial signs of Lewy body dementia can include fluctuations in cognition involving memory, following directions or ability to multitask. Difficulty paying attention in conversations is another characteristic symptom. To family and friends, this may appear to be a memory problem, Barmada said.
Visual hallucinations may occur — images that tend to be distinct to people with Lewy body.
“People often see people they know,” Barmada said. “Somebody walking in the house. Maybe they had a father or a relative that passed away, or sometimes it’s animals like a dog that they used to have.”
Before being diagnosed, people who have hallucinations are sometimes prescribed antipsychotics, which can actually make things much worse. That worsening of symptoms due to medications can be another clue, he said.
Sleep disturbances can occur years before cognitive symptoms appear. Barmada said that people with Lewy body may experience REM sleep behavior disorder, physically acting out their dreams. Maybe they’ll kick or punch, or find themselves on the floor at night.
Parkinson’s, Alzheimer’s and Lewy body connections
Lewy bodies are toxic clumps that form inside neurons from a protein called alpha-synuclein. Why the protein, which occurs in the body normally, forms these abnormal clumps is still unclear, and often doctors don’t know that someone has Lewy bodies until after they die and their brains can be examined, according to the Alzheimer’s Association.
Parkinson’s-like symptoms are often associated with Lewy body dementia and can coexist with other conditions, including Alzheimer’s, said Barmada.
“Often with Lewy body, people may be stiff. They may have falls, and it can look a lot like Parkinson’s disease, and we call that parkinsonism,” he said.
“If somebody develops the cognitive problems before the parkinsonism, they are diagnosed as Lewy body disease,” the neurologist said. “If they develop the parkinsonism first and then the cognitive problems, it’s called Parkinson’s with dementia.”
Unlike with Alzheimer’s disease, people who have Lewy body dementia often experience problems with attention, visual hallucinations and REM sleep behavior disorder along with Parkinson’s-like symptoms, Barmada said.
While the two conditions can have some overlapping symptoms, Alzheimer’s is associated with a buildup of two proteins. One is amyloid beta, and the other is called tau. Barmada said these show up as amyloid plaques and neurofibrillary tangles full of tau. That combination of amyloid and tau affects the brain regions involved in forming new memories.
Diagnosing Alzheimer’s generally involves a detailed evaluation of cognitive functioning, a neurological exam, and an assessment of medical and family history; an MRI is typically recommended as it can help rule out other issues, such as strokes, vascular conditions or bleeding in the brain.
More recently, doctors have been using a blood test that measures p-tau217, an abnormally modified version of the tau protein, and a specialized amyloid PET scan that can directly visualize amyloid buildup in the brain.
“That has changed the way that this condition is diagnosed and treated, because there are therapies that directly act on that amyloid,” Barmada said, adding that the test isn’t for otherwise healthy people, as there are other conditions that can lead to a falsely elevated level.
Having both Alzheimer’s and Lewy body can accelerate decline.
“It’s insult on injury. It just makes things worse,” he said.
Each condition has its own problems, and they compound each other. It’s becoming more evident that people who have cognitive problems late in life often don’t have just one condition, Barmada noted, though there’s a tendency to look for a single diagnosis — one condition, one answer.
“It’s very possible that with Patrick Stewart, these conditions could have been there for a very long time, but he was compensating, and it was very hard to tell until the point where it just got so bad that it started to be very clear.”
Living with Lewy body dementia
Getting a proper diagnosis is critical. Allison Feldman, CEO of the Lewy Body Dementia Association (LBDA), said that in Robin Williams’s case, he had been diagnosed with Parkinson’s disease. His diagnosis of Lewy body occurred only after his death, upon a brain autopsy.
“Had he been properly diagnosed, and knew what he was dealing with and had reasons for some of the things he was experiencing, perhaps the ending of the story might have been different,” Feldman said.
Julia Wood, director of professional and community education at the LBDA, recommends a village approach in navigating the challenges of Lewy body: social workers; psychologists; support groups (virtual ones are available); and physical, occupational and speech therapy.
She points out that fluctuations can happen moment to moment, hour to hour, day to day. “So it adds a layer of unpredictability to coping day to day.”
Things that can help:
- Slow down, Wood suggests. People with Lewy body might need 20 to 30 seconds to process information in a conversation.
- Simplify choices. For instance, don’t offer an entire menu but present two options. Offer instructions one at a time.
- Reduce distractions and consider quieter spaces for gatherings.
- Adjust the environment; if someone is experiencing hallucinations, consider a change of lighting, and remove objects that might provoke anxiety, like a coatrack with a garment that could look like a person to someone who is hallucinating.
Feldman encourages people to speak directly to people with Lewy body dementia — not to talk around them. They still want to be active, contributing members of their families and society, she said.
“I think the biggest thing I would love for people to know is that that person is still there, that individual is still there,” said Wood. “How they communicate might be different. How they respond might be different. But it’s our responsibility then to try to tailor and approach them so that we can maintain those connections.”
And Wood has a message for those living with Lewy body: “You’re not alone, and also you still have a life to live, and you need to live it with purpose and with intention.”
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