Robin Williams and Missed Signs of Lewy Body Dementia

Robin Williams was a brilliant entertainer, bringing joy and wonderment to millions of people over the past 40 years. It is always tragic when someone takes his or her own life, but if there is anything good that can be taken from such a sad event, it is to raise the public’s awareness of Lewy Body Dementia (LBD) — the most common disease that you may never have heard of before.

In recent years, Mr. Williams had been diagnosed with Parkinson’s disease and began to take medications to treat his motor symptoms in addition to medication to treat his long-standing depression. Although we will never fully understand the reasons, when Mr. Williams committed suicide, it sparked a national debate about the potential contributions of depression and Parkinson’s disease, as well as the medications used to treat these conditions, to his suicide.

The discussion broadened with the recent release of Mr. Williams autopsy findings. While the headlines initially reported the absence of illicit drugs or alcohol, more importantly the pathologist noted the findings of diffuse Lewy bodies in Mr. Williams’ brain, supporting the diagnosis of Lewy Body Dementia, the second most common cause of dementia after Alzheimer’s disease.

The Lewy Body Dementia Association (www.LBDA.org) estimates that 1.3 million Americans are affected by LBD. Symptoms of LBD include:

• A progressive dementia with prominent deficits in attention, executive function, and visuospatial abilities
• Parkinson-like features (bradykinesia, rigidity, posture, and balance)
• Visual hallucinations
• Spontaneous fluctuations in concentration and attention
• Disturbances in sleep known as REM sleep behavior disorder (i.e., acting out one’s dreams)

Although consensus criteria have excellent specificity, there is no standardized way to assess symptoms, which markedly decreases sensitivity of LBD detection and diagnosis in clinical practice.

Clinicians face a significant challenge to diagnose LBD, particularly outside of expert centers, with delays in diagnosis approaching 18 months leading to significant burden to patients and caregivers. Often patients and families must see multiple healthcare providers before a diagnosis is provided, most commonly by a neurologist.

Indeed, from reports in the news there were a number of signs that could point to LBD being the underlying case of Mr. Williams’ symptoms such as a change in mood and behavior, changes in sleep behaviors which caused Mr. Williams to have to sleep in a separate bedroom, and motor signs of Parkinson’s disease.

Depression has long been linked to an increased risk of suicide, even in individuals who are being treated with effective medications. However, there is no evidence of an inherent increased risk of suicide in individuals with LBD or other forms of dementia.

Interestingly, medications that treat the motor symptoms of Parkinson’s disease can increase risk-taking behaviors such as gambling, and may increase unusual behaviors such as hallucinations and delusions.  However, there is no strong evidence that Parkinson’s disease or the treatment of Parkinson’s disease is associated with increased suicide risk.

Early and accurate diagnosis helps families prepare for their role in caregiving, specifically behavioral management, and their own emotional preparation in anticipation of the disease course, one that may have its own unique challenges and burdens.

In a survey, LBD caregivers expressed concerns about fear for their future and the future of their loved one (77%), feelings of stress, strain, and anger (54%), interference with their social life (52%), and uncertainty about what to do next (50%).

Caregivers reported moderate to severe burden; 80% felt the people around them did not understand their burden, and 54% reported feelings of isolation, particularly in spouse caregivers. Two-thirds of the caregivers reported medical crises requiring emergency services, psychiatric care, or law enforcement.

A diagnosis of LBD should be not considered as one without hope. Many symptoms of LBD can be treated with currently available medications. In addition, patients can participate in clinical research projects to improve our understanding of LBD and potentially lead to new treatments.

Healthcare providers and advocacy groups such as the LBDA can provide effective social support for patients and caregivers to lessen perceived burden, enhance patient health, and improve quality of life.  Neurologists can take a lead role in this process by providing a comprehensive assessment of older adult cognitive, behavioral, motor, and sleep behaviors, making correct diagnoses, and initiating appropriate pharmacologic and non-pharmacologic therapies.

James E. Galvin, MD, MPH is a Professor of Neurology, Psychiatry, and Population Health, and Director of the Lewy Body Dementia Program at NYU Langone Medical Center. Dr. Galvin also serves on the Board of Directors for the Lewy Body Dementia Association.