Hispanics are at higher risk for dementia 3:28
After Robin Williams died by suicide in August 2014, his widow, Susan Schneider Williams, would soon learn of an illness she had never heard of but had haunted their lives.
That disease is Lewy body dementia (LBD), with which the actor was diagnosed in October 2014 after an autopsy on his brain.
"A few months before he passed away, he was diagnosed with Parkinson's," Schneider Williams, an artist and activist for LBD awareness and research, said at the Life Itself conference, a health and wellness event presented in partnership with CNN.
"But that was just the tip of the iceberg."
Lewy body dementia: the life-changing disease that devastated Robin Williams
The misdiagnosis occurred in May 2014 after Robin experienced severe changes in memory, movement, personality, reasoning, sleep, and mood.
The comedian underwent multiple tests to identify his problem, most of which came back negative.
"None of the doctors knew there was this phantom disease behind all of this," Schneider Williams told CNN in an interview.
"When that was revealed, it was essentially like finding out the name of my husband's killer."
Schneider Williams and Robin have dinner at a restaurant to celebrate his 50th birthday in 2014.
Dementia is a disorder of mental processes characterized by memory dysfunction, personality changes, and impaired reasoning due to brain disease or injury.
The exact cause of LBD, which affects about 1.4 million Americans, is unknown.
But the disease is associated with a buildup of the protein alpha-synuclein, which is normally present in the brain and in small amounts in the heart, muscle and other tissues.
Alpha-synuclein may help regulate neurotransmitters, but when this protein builds up and forms clumps (called Lewy bodies) inside the brain, the effects are devastating.
Lewy body dementia and Parkinson's disease dementia are the two types of Lewy body dementia, which are the second most common form of dementia after Alzheimer's disease, according to the Lewy Body Dementia Association.
Because LBD initially presents similarly to Alzheimer's or Parkinson's disease, it is often misdiagnosed.
And since Lewy body proteins can't be analyzed like Alzheimer's, LBD cases are often diagnosed after death when families request autopsies for more details or to donate a loved one's brain for research. .
Usually, for undiagnosed LBD patients who initially have movement problems, doctors first diagnose Parkinson's disease, since it is a movement disease.
If those patients also develop dementia later, they are often diagnosed with Parkinson's disease dementia.
More specific changes in cognitive function can also, over time, lead to a diagnosis of "Lewy body dementia."
Although Lewy bodies are common with Parkinson's disease, not all Parkinson's patients will develop LBD.
Misdiagnoses and overlapping symptoms can create a world of confusion for patients and their families, so for Schneider Williams, finally learning the truth behind her husband's "pain and suffering" was a "bright spot." said.
"That's when my own healing started," he said.
“We had this experience with something that was invisible and terrifying, really.
And then, on the other hand, I am left to discover the underlying science that helped explain this experience.
Robin wasn't crazy.
That was one of his biggest fears.”
So that other patients and caregivers can experience the same truth, understanding, and healing, Schneider Williams has been down a “warren of discovery” and activism for eight years.
He has served on the board of directors of the American Brain Foundation for six years, helped establish the Lewy Body Dementia Fund and its $3 million research grant to find an accurate biomarker, and contributed to the "Robin's Wish" documentaries. and "Spark: Robin Williams and her battle with Lewy body dementia."
Schneider Williams talks about LBD awareness and research at the 2021 BioHive Summit in Utah.
“I couldn't live with myself if I didn't tell this story,” said Schneider Williams.
“I had no idea of the journey that was about to begin.
But I had to go there."
Doctors and researchers who want to mitigate the kinds of experiences her husband suffered "have a difficult task," she said, "but progress is being made."
"Chemical Warfare on Your Brain"
Lewy body dementia has more than 40 symptoms that can come and go randomly, Schneider Williams said.
Categorically, the signs include thinking problems, fluctuations in attention, movement problems, visual hallucinations, sleep disturbances, behavior and mood problems, and changes in bodily functions, such as the ability to control urination.
What "marked the beginning of a cascade of symptoms" was when her husband began to experience endless fear and anxiety, Schneider Williams said.
It started happening in 2012 when Ella Williams began to stop interacting with people at the Throckmorton Theater in California, where she tried out new material and jammed with other comedians for fun, she added.
The anxieties lingered beyond what Williams had experienced in the past and beyond what is normal for a beloved actor living with the pressures of being on a world stage.
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Eventually, paranoia was another significant symptom, Schneider Williams said.
“It was the amygdala region of her brain that had a huge number of Lewy bodies.
So that area of the brain is really our ability to regulate our emotions, particularly fear and anxiety.
And Robin's was basically broken."
Towards the end, Williams also experienced delusional loops.
"Your brain is making up a story of what you think is reality," said Schneider Williams.
“And the people around you can't rationalize with you and bring you back to what's really real.
Therefore, it is incredibly scary for everyone around someone who is hallucinating and also for the person who is hallucinating.
“As a caregiver, you feel incredibly powerless when you realize, 'My God, nothing I say or do can bring him back to reality anymore.'
And that is a very scary place,” he said.
"Lewy Body: It Really Takes Over".
Williams was stressed by work, his sudden forgetfulness and changing personality, and lack of sleep, which progressed to severe insomnia that eliminated the separation between day and night in the couple's home.
“Our house was like 'Night at the Museum' at night,” said Schneider Williams.
Bringing him out of his nocturnal delusions would take hours, sometimes days, he added.
"Imagine fear on fire, that's what it is."
Hallucinations are "a key hallmark of LBD that can really help identify the disease," Schneider Williams said, but also a difficult symptom that many LBD patients don't want to discuss.
She was unaware of her husband's hallucinations until her conversation with a medical professional who had reviewed her medical records.
A delusion implies a story with people who can deconstruct it for you, but a hallucination is something that only you see and is therefore easier to hide.
“The Lewy body is neurological;
it is a circuit problem.
So chemical and structural changes in Robin's brain were responsible for the psychiatric symptoms she was experiencing,” Schneider Williams told Life Itself.
Those included depression.
The doctors Schneider Williams met with after learning of his diagnosis “indicated that his was one of the worst pathologies they had ever seen.
She had about 40% loss of dopamine neurons,” she wrote in her 2016 article “The Terrorist Inside My Husband's Brain” for the journal Neurology.
"The massive proliferation of Lewy bodies throughout his brain had caused so much damage to neurons and neurotransmitters that, in effect, you could say he had a chemical warfare going on in his brain."
The antipsychotic medications were dangerous for him and made some symptoms worse, as they do with some LBD patients, Schneider Williams said.
If people experiencing neurodegeneration can still do some routines, such as working or walking their dog, those "regular, well-worn pathways can provide comfort," Schneider Williams said.
When people can no longer do those things, symptoms can worsen and lead to devastating feelings of isolation.
"Diagnosis is everything"
Nearly eight years after the diagnosis that catalyzed Schneider Williams' investigative journey, she is "only now beginning to pick up the pieces of her own life," she said.
“I need to go underground for a while and relocate my inspiration and true passion, which is art and painting,” said Schneider Williams.
She plans that a portion of all future print sales of hers will go towards LBD research, and she will stay in touch with documentary-related efforts and the Lewy Body Dementia Fund, where she remains the senior president.
Schneider Williams paints at his home in Marin, California, in 2019.
As Schneider Williams widens his focus and leaves the door open for the LBD defense, experts continue their investigative efforts.
“We are always learning more and more about the disease, from basic science studies looking at cells and test tubes, to animal models and observational studies in humans,” said James Galvin, MD, professor of neurology and director of the Center. Comprehensive Brain Health Program at the University of Miami Miller School of Medicine.
Recent highlights include the introduction of at least two new diagnostics, Galvin said: a cerebrospinal fluid test from the company Amprion and a skin biopsy test from CND Life Sciences.
The cerebrospinal fluid test tracks misfolded synuclein and helps doctors diagnose brain diseases, including LDL.
The skin biopsy test is intended to help doctors distinguish between serious neurological disorders.
"Having diagnoses, which can confirm in life that someone has Lewy body disease, is very helpful both in confirming the diagnosis and in advancing research," Galvin said.
"The earlier you can start treatments, the easier it will be to enroll people in clinical trials to test new drugs."
The National Institutes of Health awarded Galvin and the company Cognition Therapeutics a $29 million grant to study whether a new drug, CT1812, is safe and effective for patients with LBD.
To treat LBD, doctors "borrow drugs from Alzheimer's to treat cognitive symptoms, from Parkinson's to treat motor symptoms, from narcolepsy to treat attention deficits, and from psychiatry to treat behavioral symptoms," Galvin said in a press release.
CT1812 could help the brains of LBD patients clear toxic proteins and protect against functional loss.
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"When I wrote that 'The Terrorist Inside My Husband's Brain' editorial, I was convinced that a diagnosis wouldn't matter anyway, because there is no cure," Schneider Williams told Life Itself.
“But my way of thinking since then has completely changed.
Diagnosis is everything, not only for patients and caregivers, but also for physicians, clinicians, and researchers.
If we had an accurate diagnosis, we could have sought specialized care.”
The Lewy Body Dementia Association has formed a Research Centers of Excellence Program, with 22 sites across the United States, to collaborate on clinical trials, assess resource and infrastructure needs, and develop better measures of clinical symptoms, Angela said. Taylor, interim director of the association.
"We can't undo changes that have already occurred," said Samantha Holden, an associate professor of neurology at the University of Colorado and director of the Memory Disorders Clinic at UCHealth Hospital at the University of Colorado.
"But if we catch people early enough, can we stop it from progressing?"
Research progress is being made bit by bit.
When asked if she ever felt like giving up, Schneider Williams said: “Oh my God.
Pick a day."
“It's very overwhelming when you look at all the millions and billions of dollars being spent on research and you think, 'My God, have we really made any progress?'” he added.
But as complex as LBD is, "every yard gained matters."
"Whoever has hope has many days to feel the darkness," said Schneider Williams.
“But the thing about hope is that whatever happens, you dust yourself off, get up and move on.
And you don't do that alone."
Call 1-800-273-8255 in the US to contact the National Suicide Prevention Lifeline. Provides free and confidential assistance 24 hours a day, seven days a week, for people in suicidal or distressed crisis. You can learn more about their services here, including their guide on what to do if you spot suicidal signs on social media. You can also call 1-800-273-8255 to speak with someone about how you can help someone in crisis. Call 1-866-488-7386 for TrevorLifeline, a suicide prevention counseling service for the LGBTQ community.
Call 1-800-273-8255 in the US to contact the National Suicide Prevention Lifeline.
Provides free and confidential assistance 24 hours a day, seven days a week, for people in suicidal or distressed crisis.
You can learn more about their services here, including their guide on what to do if you spot suicidal signs on social media.
You can also call 1-800-273-8255 to speak with someone about how you can help someone in crisis.
Call 1-866-488-7386 for TrevorLifeline, a suicide prevention counseling service for the LGBTQ community.
For assistance outside the US, the International Association for Suicide Prevention provides a global directory of resources and international hotlines.
You can also turn to Befrienders Worldwide.
See here where to look for help in Latin American countries and Spain.