What exactly is Lewy Body Dementia, the disease that killed Robin Williams?
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Robin Williams tickled our collective funny bone for more than four decades with his manic, subversive humour. But nobody was laughing when the actor took his own life in August 2014, at the age of 63.
His family, friends and fans around the world struggled to understand how a talented, rich, successful family man could find life so unbearable that he had to end it all. At first, rumour circulated that he had lost his long battle with alcohol and drug abuse. That was soon supplanted with reports the funny man suffered from severe depression. Then surfaced news that he’d been diagnosed with Parkinson’s disease. Last week his widow, Susan Schneider Williams, opened up about the real reason her husband committed suicide. “It was not depression that killed Robin,” she told People. “Depression was one of, let’s call it, 50 symptoms, and it was a small one.”
What really drove the comedian to take his life was a little-known disease called Lewy Body Dementia, also referred to as Dementia with Lewy Bodies. Characterized by anxiety, depression, hallucinations and loss of motor function, among other symptoms, LBD is often misdiagnosed as Parkinson’s, as it shares some of that disease’s markers.
To shed light on this puzzling and devastating disease, we spoke with Dr. Mario Masellis, Clinician-Scientist and Assistant Professor in the Department of Medicine (Neurology) at the University of Toronto’s Sunnybrook Health Sciences Centre.
What is Lewy Body Dementia?
Dr. Masellis says he was not surprised by Schneider Williams’s statements that her husband had initially been diagnosed with Parkinson’s disease and an autopsy found Lewy Body Dementia. “Parkinson’s disease with dementia and Dementia with Lewy Bodies fall under an umbrella of diseases that are known as Lewy Body Spectrum Disorders,” he says. “These are actually not different diseases, they are highly related.”
Dr. Masellis explains that Lewy bodies, named after Frederic Lewy, the American neurologist who discovered the abnormal protein deposits in the early 1900s, are small inclusions within brain cells containing a protein called alpha-synuclein. We need this protein for our brains to function properly. But as we age, the protein becomes sticky and clumps together, damaging brain cells in those of us who may be predisposed.
With Parkinson’s disease, the Lewy bodies affect mainly the motor system in early stages of the disease, resulting in tremors, stiffness, rigidity and slowness of movement. With LBD, the Lewy bodies wreak havoc with our motor system as well, but they also invade other areas of the brain, including the limbic system, which controls behaviours and emotions, as well as the cortex, which governs our ability to think and reason. The pathology is the same with both diseases: the Lewy bodies cause the death of brain cells. However, in patients with Parkinson’s, this pathology may spread over time as the disease progresses to also involve the limbic system and cortex, resulting in the same symptoms as LBD.
Robin Williams and his wife in 2012 (Getty Images)
What are the symptoms?
Not exactly the 50 that Schneider Williams mentioned, although it can seem like it when the patient is afflicted with memory and cognitive problems, trouble with attention and alertness, motor difficulty, depression, difficulty performing day-to-day functions, anxiety, vivid visual and auditory hallucinations and paranoia. As his widow says, the comedian felt he was losing his mind.
How common is it?
Dr. Masellis says LBD, when combined with Parkinson’s dementia, is the second-most common form of neurodegenerative dementia after Alzheimer’s disease. He says there are no definitive studies documenting the number of Canadians living with the disease (about one million Americans are affected) and only in rare cases is it strongly hereditary.
However, in one study, Lewy body changes accounted for about 20 per cent of dementia cases autopsied. Males are more likely to be afflicted, and the biggest risk factor is aging, which means it’s on the rise.
How is it diagnosed?
Like Alzheimer’s disease, LBD can only be absolutely diagnosed after death, but, as Dr. Masellis says, “If you have the right combination of symptoms you have a pretty high predictive value that you’re going to find Lewy bodies in the brain.”
Researchers don’t know what causes LBD, and, according to the Alzheimer Society of Canada, there are no known risk factors. The Society says annual spending on dementia overall is expected to increase from today’s $33 billion to $239 billion by 2040.
How is it treated?
Dr. Masellis says, despite ongoing research and experimental studies, a cure is a long way off. In the meantime, current therapy includes cholinesterase inhibitors helpful for hallucinations and cognitive impairment, and levodopa that can help with motor problems. On occasion, two atypical anti-psychotic drugs— Quetiapine or Clozapine — can be prescribed to treat the psychotic symptoms, such as the hallucinations and delusions, in cases not responding to cholinesterase inhibitors.
Robin Williams with his daughter Zelda in 2011 (Getty Images)
What’s the prognosis?
“There are variants of these diseases, more severe and aggressive and less severe and aggressive, so I would find it surprising that someone could say with certainty that you would only have three years. That’s highly unusual,” he says of the time frame Robin Williams was reportedly given to live. “I generally tell my patients that it’s a progressive disease; it gets worse over time. Some people have a more slowly progressive course, some more quickly.”
Dr. Masellis will say, however, that “there comes a point in the course of all neurodegenerative diseases where we can say that [the patient] will likely have six months to a year to live. But this is when they have come to the point where they have difficulty swallowing or are completely immobile, and have other morbidities associated with the disease.”
On average, he says, the prognosis ranges from the time the symptoms start to the time they pass away at about six to 10 years.
Can it be prevented, slowed or reversed?
While he wouldn’t comment on the subject of Robin Williams’s well-documented history of drug and alcohol abuse having any correlation to his development of the disease, Dr. Masellis did say, “if you have other vulnerabilities such as cerebral vascular disease or stroke, it may lower your threshold to be able to compensate for neurodegenerative pathologies like Alzheimer’s or LBD. Someone who does not have hypertension, diabetes and high cholesterol and develops Parkinson’s disease or LBD — and this is only theoretical, I don’t think studies have been done — they may be less likely to develop Parkinson’s dementia than someone who does have uncontrolled cardiovascular risk factors.”
In the end, Dr. Masellis dispenses the same advice you so often hear like a mantra for any kind of disease prevention: exercise and a healthy lifestyle. Advice worth remembering.
