Many people had never heard of Lewy body dementia until last month, when actor Robin Williams’ wife first spoke publicly about his struggle with the disease before he committed suicide in August 2014.
“Lewy body dementia killed Robin,” Susan Williams told an ABC News interviewer in early November. “It’s what took his life.”
The disease is obscure despite its prevalence. Lewy body dementia afflicts an estimated 1.3 million people in the United States alone, and experts say it’s the second or third most common neurodegenerative disorder after Alzheimer’s disease.
Often confusing it with Alzheimer’s or Parkinson’s disease, with which it shares symptoms, medical professionals and lay people alike have been slow to understand Lewy body dementia as a distinct ailment with its own set of symptoms and specialized treatments.
“It’s underrecognized,” said Shawn Kile, a neurologist and director of Sutter Health’s Neuroscience Institute Memory Clinic. “The public doesn’t know about it that much, and even some physicians aren’t familiar with the diagnosis.”
Kile said his clinic sees about 50 new Lewy body patients a year, mostly in their 60s, 70s and 80s.
Robin Williams was initially diagnosed with Parkinson’s disease because of the muscle rigidity common to both conditions. Others with Lewy body dementia are diagnosed with Alzheimer’s because of memory loss and impaired cognitive abilities.
Lewy body dementia – also called dementia with Lewy bodies or diffuse Lewy body dementia – can first show effects similar to those of other brain disorders, but it has its own set of hallmark symptoms, including paranoia, severe anxiety and odd behavior, all of which Williams reportedly experienced.
One of its most distinctive symptoms is hallucinations. Sufferers will often see animals or people that aren’t there. Many hallucinations are benign, but some are frightening, such as house fires.
Joyce Farruggia said her late husband, Joseph Farruggia, saw mostly friendly apparitions before he died in September 2013 at age 84.
“He saw things from his childhood, like his sister who died when he was 12,” she said. “His brother (who had died) would sit on the bed with him and give him comfort. Luckily his hallucinations were friendly.”
Joseph Farruggia, a longtime music professor at Humboldt State University, showed symptoms of unusual behavior, such as giving a 20-mile car ride to a woman he didn’t know and buying “lots and lots of pens,” his wife said.
Robin Williams, shortly before he died, stuffed a number of wristwatches in a sock and gave them to a friend for safekeeping, according to a death investigation. His wife said in interviews he’d experienced increasing paranoia in the weeks before his death.
The “Lewy bodies” that cause such behavior are abnormal protein deposits in the brain, similar to those that cause Parkinson’s disease. They are named for researcher Friederich Lewy, who discovered them in the early 1900s.
In Parkinson’s, the deposits are primarily concentrated in the midbrain and interfere with physical functioning first. In Lewy body dementia, the deposits are spread throughout the brain, including areas that control thinking, memory and behavior.
“The classic dementia with Lewy bodies presents with changes in motor skills and thinking that are often simultaneous,” said Charles DeCarli, director of the Alzheimer’s Disease Center at UC Davis Medical Center in Sacramento.
Lewy body dementia can make people who are asleep act out as if they’re awake, he said.
“They may be having a vivid dream in which they believe they’re playing football or they’re back in Vietnam,” DeCarli said. “They thrash about and fall off the bed or strike their partner.”
Like Alzheimer’s, Lewy body dementia generally affects older people, doctors said. More men than women seem to be afflicted. And the disease, which progressively destroys brain matter over a period of years, is ultimately fatal.
“As the disease progresses, it affects more and more brain cells,” Kile said. “You lose neurological functions – the drive to eat, the ability to protect your airway when you swallow. It’s those things that eventually cause death.”
Patients typically live about eight years after they are diagnosed, the neurologist said.
They remember they were a very intelligent person and they can’t do the things they used to. It’s very depressing for them.
Bernie Ford, whose husband died of Lewy body dementia
As the disease takes hold, cognitive function can come and go, meaning that patients can have days when they seem like their old selves and other days in which delusions, paranoia and anxiety alter their personalities.
“It can be emotionally difficult for the caregiver,” said Denise Davis, a program director with the Alzheimer’s Association and facilitator of the Lewy body dementia support group for the Sacramento area. (The group meets once a month at the Eskaton Lodge assisted-living facility in Gold River. Contact Denise Davis at firstname.lastname@example.org or (916) 930-9080.)
“The fluctuations (in cognitive ability) are more pronounced in Lewy body dementia than in Alzheimer’s,” Davis said. “It’s more of a progression with Alzheimer’s and not a roller coaster.”
Bernie Ford said her late husband, Lee Ford, who taught computer programming at American River College in Sacramento, was diagnosed with Parkinson’s disease in 2001 before doctors changed their diagnosis to Lewy body dementia in 2005. He died in 2011.
Lee Ford was a quiet, learned man who enjoyed teaching, his wife said. Like other Lewy body patients, his deterioration was particularly troubling because at times he would regain briefly the faculties he’d lost, she said.
“It’s frustrating for them because at moments they realize who they were,” Bernie Ford said. “They remember they were a very intelligent person and they can’t do the things they used to. It’s very depressing for them.”
The difficulty and slowness of diagnosing the disease are other frustrations, caregivers said.
Many patients, such as Williams, are initially diagnosed with Parkinson’s. Though Williams had displayed classic Lewy body symptoms of paranoia and anxiety, it wasn’t until his autopsy, when pathologists could see the protein deposits clearly, that they realized his brain was riddled with Lewy bodies.
“We’ve got to get better at diagnosing Lewy body dementia,” Davis said.
Appropriate treatments depend on accurate diagnoses. For instance, medications given to Lewy body patients replace a brain chemical that the disease severely depletes, experts said.
Some Parkinson’s patients, in contrast, receive treatments that if wrongly administered can worsen symptoms of Lewy body dementia, according to the National Institute on Aging.
“LBD-related movement symptoms may be treated with a Parkinson’s medication … (that) can help improve functioning by making it easier to walk, get out of bed, and move around,” the NIA says on its website. But “side effects of this medication can include hallucinations and other psychiatric or behavioral problems.”
Robin Williams took his own life before doctors were able to accurately diagnose him. But whether the disease caused him to kill himself is a controversial notion among experts.
“Robin was very aware that he was losing his mind and there was nothing he could do about it,” Susan Williams told People magazine, explaining his suicide.
Depression is common among those with dementia and Parkinson’s. But both Kile, at Sutter, and DeCarli, at UC Davis, said they’d never treated a Lewy body patient who committed suicide.
“In almost 30 years, I’ve never seen a suicide in a Parkinson’s or LBD patient,” DeCarli said. “I haven’t seen suicides in Alzheimer’s patients either. Robin Williams’ wife said LBD killed him. I think that’s an overstatement.”
Bernie Ford said she understood Susan Williams’ thinking and that it “rang true” to some degree, but Ford said she felt Williams might have been reaching for an explanation for her husband’s suicide.
Joyce Farruggia said elements of what Williams said in interviews also made sense to her. Her husband became depressed when he could no longer play the flute or socialize with friends as he once did, she said.
In Robin Williams’ case, it sounded as though Lewy body dementia had robbed him of his rapid-fire wit and acting ability, she said.
“He was aware of what was happening to him,” Farruggia said. “To not be able to do the things you used to do is very depressing.”