In the wake of Susan Williams’ revealing interview about what drove her husband Robin Williams to commit suicide last August – that Williams did not suffer from major depression, but rather a commonly misdiagnosed brain disease called Lewy Body Dementia (LBD) or Dementia with Lewy bodies (DLB) – Susan’s heart wrenching details about how she and Williams struggled for more than a year to figure out what was wrong with him begs the question: What exactly is LDB? And why is it so difficult to diagnose?
Caused by abnormal protein deposits that build up over time and disrupt normal brain function, the disease is particularly challenging to diagnose because its laundry list of symptoms varies from person to person.
“Patients can have different presentations – some patients start off with memory and thinking problems, particularly in their visual-perceptive abilities,” says Dr. James E. Galvin, Associate Dean for Clinical Research at Florida Atlantic University and Director of the Leon and Toby Cooperman Center for Alzheimer Disease and Memory Disorders at the Marcus Neuroscience Institute in Boca Raton, Florida.
“Others start off with movement problems: slowed movements, unsteady walking, falls. Still others have changes in their behavior: anxiety and depression with a loss of interest in things around them (known as apathy) and may start to experience visual hallucinations (usually small people or animals) or have false beliefs (delusions) such as their loved ones being replaced by imposters.”
Adding to that difficulty in diagnosis is the fact that many of the symptoms are consistent with other serious conditions such as Parkinson’s, Alzheimer’s and depression, leading to misdiagnosis.
“DLB is often misdiagnosed as Parkinson’s disease, like in Robin’s case,” says Dr. Alex Pantelyat, Director of Atypical Parkinsonism Center and Assistant Professor of Neurology at Johns Hopkins. “Sometimes as Alzheimer’s disease or another disorder depending on what symptoms predominate, if they are cognitive or movement-related.”
Even more troubling than the misdiagnoses is the fact that an estimated 1.4 million people are suffering from the disease and might not know it.
“DLB is the second most common neurodegenerative dementia after Alzheimer’s,” says Pantelyat. “It is certainly not rare, it is the most common of the atypical Parkinsonian disorders that I deal with. That coupled with lack of recognition adds up to a lot of patients who are not getting the appropriate care.”
Dr. Galvin adds that the general lack of awareness and standardized testing for the disease only complicates the issue.
“Even though the symptoms of LBD are present in the majority of patients, it is difficult to come up with a standardized way to assess each of the symptoms so that the average physician can easily make a diagnosis,” he says. “This leads to significant delays – up to 18 months with most patients seeing multiple doctors before a diagnosis is established.”
When a diagnosis does not come quickly, Dr. Pantelyat says patients who are in the early stages of the disease have a higher risk of self-harm.
“We very well know that patients with depression in general are much more likely to have attempts at suicide,” he says. “With regard to atypical Parkinsonian disorders including DLB, depression is very highly comorbid [meaning it very often exists with or alongside another medical condition]. I would argue that in the milder earlier clinical stages of disease is when the risk for suicide would be highest because from a movement perspective people are less impaired, meaning they can actually do things about it. It’s incredibly devastating but I’ve had patients tell me about suicidal ideation but they have the full realization that they can’t do anything even if they wanted to because they’re so limited from a movement perspective.”
No matter how you look at or understand it, Lewy Body Dementia is an “incredibly devastating disease,” continues Pantelyat. “It affects your core, it affects who you are as a person. In the case of DLB and some of these other related disorders it tends to a great extent affect the frontal lobe, which is really what makes us human. It’s really unbelievably devastating. Very often people don’t have insight into what’s going on, it’s the caregiver that has to bear that burden, which is incredible. One of the things I would sorely want to see as soon as possible as a result of rising awareness would be more groups formed, both online and community-based. Support groups for spouses and caregivers for these patients. This is a great need. People are not being diagnosed.”
For much more from Susan Williams, pick up the new issue of PEOPLE, on newsstands Friday