Saturday, January 29, 2022 – Lewy Body Dementias
- Mary Reed
- Jan 29, 2022
- 8 min read
This year would have been Robin Williams’ 70th birthday. So, there has been publicity about him. I always thought he was high-strung, living on the edge, and he fell off; that’s why he committed suicide. But recently I learned that he was diagnosed with Lewy body dementia from his autopsy. In fact, there was a documentary — “Robin’s Wish” — made about it in late 2020. I guess I must have been under a rock for the last year and a half, but I do not remember hearing about it. It was such a tragic end for a wildly brilliant performer. I have a friend whose husband had Lewy body dementia, along with other illnesses, before he died. It can be very stressful for the caregiver. She had to take away his car keys because he couldn’t be trusted driving. Lewy body dementias are difficult and not well-known diseases. Let’s learn more about them.
Lewy body dementias are two similar and common subtypes of dementia — dementia with Lewy bodies or DLB and Parkinson's disease dementia or PDD. Both are characterized by changes in thinking, movement, behavior and mood. The two conditions have similar features and may have similar causes, and are believed to belong on a spectrum of Lewy body disease that includes Parkinson's disease. As of 2014, they were more often misdiagnosed than any other common dementia.
The exact cause is unknown but involves widespread deposits of abnormal clumps of protein that form in neurons of the diseased brain. Known as Lewy bodies — discovered in 1912 by Frederic Lewy — and Lewy neurites, these clumps affect both the central nervous system and the autonomic nervous system. The fifth revision of the Diagnostic and Statistical Manual of Mental Disorders gives Lewy body disease as the causative subtype of dementia with Lewy bodies, and Parkinson's disease as the causative subtype of Parkinson's disease dementia. Dementia with Lewy bodies is marked by the presence of Lewy bodies primarily in the cortical regions, and Parkinson's disease dementia with Lewy bodies primarily in the subcortical basal ganglia.
Classification
The synucleinopathies — dementia with Lewy bodies, Parkinson's disease dementia and Parkinson's disease — are characterized by shared features of parkinsonism motor symptoms, neuropsychiatric symptoms, impaired cognition, sleep disorders and visual hallucinations. The Lewy body dementias — dementia with Lewy bodies or DLB) and Parkinson's disease dementia or PDD — are distinguished by the timing when cognitive and motor symptoms appear. The two Lewy body dementias are often considered to belong on a spectrum of Lewy body disease that includes Parkinson's disease.
Medical subject headings list Lewy body disease in several categories: as a nervous system disease in two listings — one as a basal ganglia Parkinsonian movement disorder and the other under brain disease as a dementia, as a neurodegenerative disorder listed as a synucleinopathy and as a neurocognitive disorder listed with dementia.
Cause and mechanisms
Dementia with Lewy bodies and Parkinson's disease dementia are similar in many ways, suggesting there may be a common pathophysiological mechanism with PDD and DLB at opposite ends of a Lewy body disease spectrum, and a shared component of protein deposits in Lewy bodies and Lewy neurites. Lewy bodies and neurites have been found to develop from the aggregation of misfolded alpha-synuclein, a protein thought to assist in neurotransmitter release and vesicle turnover. Whether these misfolded proteins are responsible for the neurodegenerative effects remains unclear, and no definitive link between Lewy bodies and neurodegenerative effects has been found. DSM-5 — fifth revision of the Diagnostic and Statistical Manual of Mental Disorders — gives Lewy body disease as the causative subtype of DLB, and Parkinson's disease as the causative subtype of PDD. DLB is marked by the presence of Lewy bodies primarily in the cortical regions, and PDD with Lewy bodies primarily in the subcortical basal ganglia.
Despite differences in the timing of the appearance of symptoms, the two dementias "show remarkably convergent neuropathological changes at autopsy." The relationship between Parkinson's disease dementia and dementia with Lewy bodies is unclear as of 2020, but there is likely to be genetic overlap, and the two conditions may represent different points on a continuum.
Diagnosis
Dementia with Lewy bodies and Parkinson's disease dementia have similar neuropathological features, but these features are highly variable, and the conditions cannot be distinguished on pathological features alone. Generally, dementia with Lewy bodies is distinguished from Parkinson's disease dementia by the time frame in which dementia symptoms appear relative to parkinsonian symptoms and is diagnosed when cognitive symptoms begin before or at the same time as parkinsonism. Parkinson's disease dementia is the diagnosis when Parkinson's disease is already well established before the dementia occurs.
Epidemiology
Between 5% and 25% of diagnosed dementias in older adults are due to one of the Lewy body dementias. As of 2014, the Lewy body dementias affect about 1.3 million people in the U.S. and 140,000 in the UK.
LBD usually develops after the age of 50. Men are more likely to be diagnosed than women.
Prognosis
Life expectancy of people with one of the LBDs is reduced; following diagnosis, it ranges on average from five to eight years.
Society and culture
Advocacy and awareness
As of 2014, the Lewy body dementias were more often misdiagnosed than any other common dementia. Most people with DLB had not heard of the condition prior to diagnosis; general awareness about LBD lags well behind that of Parkinson's and Alzheimer's diseases, even though LBD is the second most common dementia after Alzheimer's. It is not only frustrating for families and caregivers to find that few people — including many health care professionals — are knowledgeable about LBD; lack of knowledge can have significant health consequences because people with LBD have severe sensitivity to antipsychotics often used to treat the symptoms. The Lewy Body Dementia Association and the UK Lewy Body Society promote awareness and provide support that helps society, by reducing costly use of health care and reducing stress for families with LBD. These organizations and others in Argentina, Australia and Japan help increase knowledge and help families with LBD become advocates to raise awareness about the disease.
Notable individuals
Robin Williams, the American actor and comedian, died on August 11, 2014. Before his suicide, he had been diagnosed with Parkinson's disease, and according to his widow — Susan Schneider Williams — he experienced depression, anxiety and increasing paranoia. Upon autopsy, his widow said he was found to have diffuse Lewy body disease, while the autopsy used the term diffuse Lewy body dementia. The vocabulary associated with Lewy pathology causes confusion. Lewy body dementia — the umbrella term that encompasses the clinical diagnoses of dementia with Lewy bodies and Parkinson's disease dementia — differs from Lewy body disease, the term used to describe pathological findings of Lewy bodies on autopsy. Dennis Dickson, a spokesperson for the Lewy Body Dementia Association, clarified the distinction by stating that diffuse Lewy body dementia is more commonly called diffuse Lewy body disease and refers to the underlying disease process. According to Dickson, "Lewy bodies are generally limited in distribution," while in dementia with Lewy bodies, "the Lewy bodies are spread widely throughout the brain, as was the case with Robin Williams." Ian G. McKeith, professor and researcher of Lewy body dementias, commented that Williams' symptoms and autopsy findings were explained by dementia with Lewy bodies.
The British author and poet Mervyn Peake died in 1968 and was diagnosed posthumously as a probable case of DLB in a 2003 paper published in JAMA Neurology. Sahlas said his death was "variously ascribed to Alzheimer disease, Parkinson disease or postencephalitic parkinsonism." Based on signs in his work and letters of progressive deterioration, fluctuating cognitive decline, deterioration in visuospatial function, declining attention span and visual hallucinations and delusions, his may be the earliest known case where DLB was found to have been the likely cause of death.
Other entertainers and artists who have or died from LBD include Estelle Getty, the actress known for her role as Sophia on the television series “The Golden Girls;” Nicholas King, a U.S. actor and horticulturist; actress Dina Merrill; Donald Featherstone, who created the plastic pink flamingo; American radio and television host Casey Kasem; Canadian singer Pierre Lalonde; graphic artist/film set designer Ron Cobb; American actor Frank Bonner; and Canadian musician and actor André Gagnon.
Individuals from industry or government who have died from LBD are Seymour Berry, U.S. director of the Bureau of Engraving and Printing; Los Angeles Times publisher Otis Chandler; Philip J. Rock, a U.S. Democratic politician of the Illinois Senate; and U.S. media mogul and philanthropist Ted Turner.
Arnold R. Hirsch, an American historian who taught at the University of New Orleans, and Jessie Isabelle Price, an American veterinary microbiologist, also died from LBD.
In the sports realm, Jerry Sloan, American professional basketball player and coach, died from LBD. Major League Baseball players Tom Seaver, Andy Carey and Bill Buckner died of LBD. Stan Mikita, Canadian ice hockey player, was diagnosed with possible LBD, but a post-mortem brain autopsy found that he had chronic traumatic encephalopathy.
Robin’s Wish
“Robin's Wish” is a 2020 American documentary film directed by Tylor Norwood. The film offers a look into the life and final days of Robin Williams and how his struggles with diffuse Lewy body disease impacted his acting career and contributed to his death by suicide. The film was produced by Ben Sinclair, executive produced by physician and journalist Shoshana R. Ungerleider and released digitally and on-demand on September 1, 2020.
Robin Williams' widow, Susan Schneider Williams, has called Lewy body dementia “the terrorist inside my husband’s mind.” She chose the film's title and approached director Tylor Norwood to make the film. Initially resistant, Norwood said he wanted to help people "understand the pain [Williams] felt as his talents and faculties rapidly slipped away," and he hoped the film "rights a wrong that was done to him,and takes away a cloud that has unjustly hung over his legacy for far too long." Interviews include Susan Williams and Shawn Levy — who directed Williams in the “Night at the Museum” series — as well as John R. Montgomery and David E. Kelley, who both worked with Williams on the TV series “The Crazy Ones.”
On the review aggregator website Rotten Tomatoes, the film holds an approval rating of 89%, based on 57 reviews, with an average rating of 7.3/10. The site's critics consensus reads: "’Robin's Wish’ offers an emotional look at the end of a life suddenly cut short — and pays warm tribute to the brilliant legacy that was left behind." On Metacritic, the film has a weighted average score of 78 out of 100, based on 11 critics, indicating "generally favorable reviews."
Frank Scheck of The Hollywood Reporter called the film "a moving coda to a life ended much too soon" and wrote: "’Robin's Wish’ suffers at times from its lack of objectivity. Williams' widow is clearly the project's driving force, with much of the story told from her perspective; his children, on the other hand, are not heard from at all. Nonetheless, the film, which incorporates testimony from several medical experts, fully succeeds in its admirable goal of using Williams' story to shed light on a disease with which many people were previously unfamiliar."
SPARK: Robin Williams and his Battle with Lewy Body Dementia
From the movie “Robin’s Wish,” the Lewy Body Dementia Association and the producers of “Robin’s Wish” created a 45-minute documentary adaptation, “SPARK: Robin Williams and his Battle with Lewy Body Dementia.” The film highlighted key messages that can enhance learning and understanding of LBD, improve diagnosis and detection and offer LBD families a way to feel connected to resources and support.
Sleepwalk with Me
“Sleepwalk with Me” is a 2012 American independent comedy film written by, directed by and starring Mike Birbiglia. It also stars Lauren Ambrose, Carol Kane, James Rebhorn and Cristin Milioti. The film had its world premiere at the Sundance Film Festival on January 23, 2012, where it won the Best of NEXT Audience Award. It was released on August 24, 2012, by IFC Films. The story featured in the first act of episode #361 of NPR radio program “This American Life,” titled "Fear of Sleep."
Based on a true story as told in his one-man off-Broadway show and his first book, the film follows the journey of an aspiring comedian in denial about the fate of his relationship, his goals for the future and his emergent rapid eye movement sleep behavior disorder. The longer his feelings of anxiety go unexpressed, the more uproarious and dangerous his sleepwalking incidents become.
In 2008, Birbiglia wrote and starred in “Sleepwalk with Me,” a one-man show performed off-Broadway at the Bleecker Street Theatre in New York City. The show blended stand-up comedy with theater and was a critical success. The New York Times called the Nathan Lane-presented show "simply perfect" and Time Out New York named it their "Show of the Year" for 2009.
After his theatrical debut, Birbiglia wrote “Sleepwalk With Me & Other Painfully True Stories.” The book — which debuted at No. 29 on the New York Times' hardcover nonfiction bestseller list — was based on his one-man show of the same name and was a finalist for the 2011 Thurber Prize for American Humor.
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