Susan Schneider Williams addresses ANA Presidential Symposium; Recounts struggle to diagnose late husband’s Lewy body dementia


Newswise — In the fall of 2013, Robin Williams began suffering symptoms of a disease whose name he would never know but would cause him to take his life in August 2014.

“I felt like I was watching my husband disintegrate before my eyes and there was nothing I could do about it,” said Susan Schneider Williams, widow of the late actor and comedian told 1,000 neurologists and neuroscientists at the American Neurological Association’s 143rd Annual Meeting.

As the keynote speaker for the Presidential Symposium, “Lewy Body Dementia (LBD): From Symptoms to Synuclein,” Williams put a human face on the leading-edge science presented.

LBD is the second most common type of degenerative dementia, yet little is understood about its pathogenesis including the cause of synuclein accumulation in the cerebral cortex, its spread through the brain, how synuclein leads to neuronal dysfunction, and the relationship of LBD to Parkinson’s disease. Symptoms are varied, ranging from hallucinations to digestive issues, and can be fleeting, appearing for months at a time only to quickly disappear and be replaced by new ones.

After more than a year of devastating physical and psychological symptoms that included severe anxiety, paranoia, and movement dysfunction, Robin Williams’ medical team arrived at a Parkinson’s disease diagnosis. A few months later, “exhausted from the terror coming from his brain, Robin took his life before it could take more of him,” Williams said.

The coroner’s report revealed the true source of Robin Williams’ illness: LBD, one of the most extreme cases the physicians had ever seen. “Robin had diffuse LBD throughout his brain and brain stem, with a concentration in his amygdala, as well as amyloid plaques and tau tangles. I used to tell people that he had a dementia cocktail,” Williams said.

While some of the nearly 40 symptoms that characterize LBD are common, such as anxiety, sleeplessness, and depression, “the challenge to diagnosing and ending LBD is seeing it as the giant constellation it is, rather than a few of its stars,” Williams explained.

Research presented at the symposium offered hope that effective therapies for LBD are on the horizon.

“The ultimate goal is to use genetics and biomarkers to identify patients at risk of development Lewy body dementia and provide them with targeted treatments. What we’re working toward is catching patients in the early symptomatic stages so that we can develop more effective therapies,” said Dimitri Krainc, MD, PhD, Northwestern University Feinberg School of Medicine, and 2018 ANA Soriano Lectureship Award recipient.

Since her husband’s death, Williams has been a committed advocate for LBD research and awareness.  She has traveled to Washington to educate policymakers on the need for funding to better understand this elusive disease, and serves as vice chair of the American Brain Foundation, which provides grants to support research on neurodegenerative diseases.

“I believe that Robin’s death and his battle against these diseases holds a profound purpose. There was power in what he suffered. I’m here to see that power transformed into something good.”

Renowned academic neurologists participating in the Presidential Symposium and their topics include:

Chair: David M. Holtzman, MD (Washington University in St. Louis)

Co-Chair: William T. Dauer, MD (University of Michigan)

Bradley F. Boeve, MD (Mayo Clinic): Clinical, Diagnostic and Prognostic Features of LBD

Dmitri Krainc, MD, PhD (Northwestern University): The Interplay of Mitochondria and Lysosomes in Neurodegeneration

Virginia Man-Yee Lee, PhD (University of Pennsylvania): Cell-to-Cell Transmission of Misfolded Alpha-Synuclein as a Disease Mechanism in Lewy Body Dementia/Parkinson Dementia

Susan Schneider Williams, the widow of Robin Williams, shared her perspective on living with someone affected by LBD

In addition, brief “Data Blitz” presentations highlighted additional findings including:

Rakez Kayed, PhD (University of Texas Medical Branch): Propagation of Brain Derived Disease Specific Tau Oligomeric Strains via the Eye-Brain Axis

Urmi Sengupta, MS (University of Texas Medical Branch): The Role of α-Synuclein Strains on Tau Aggregation in Disease Pathology Urmi Sengupta, MS, University of Texas Medical Branch

Anthony Downs, BS (Emory University): Nicotinic Receptor Dysfunction Underlies Abnormal Responses to Muscarinic Receptor Antagonist Treatment in a Mouse Model of DYT1 Dystonia

The ANA Annual Meeting convenes 1,000 of the nation's top academic neurologists and neuroscientists to share updates and late-breaking research on the diseases that affect more than 100 million Americans each year including stroke, Alzheimer’s disease, Parkinson’s disease, traumatic brain and spinal cord injuries, epilepsy, multiple sclerosis and more. Review the complete program here.

About the American Neurological Association (ANA)

From advances in stroke and dementia to movement disorders and epilepsy, the American Neurological Association has been the vanguard of research since 1875 as the premier professional society of academic neurologists and neuroscientists devoted to understanding and treating diseases of the nervous system.  Its monthly Annals of Neurology is among the world’s most prestigious medical journals, and the ANA’s Annals of Clinical and Translational Neurology is an online-only, open access journal providing rapid dissemination of high-quality, peer-reviewed research related to all areas of neurology.  The acclaimed ANA Annual Meeting draws faculty and trainees from the top academic departments across the U.S. and abroad for groundbreaking research, networking, and career development.  For more information, visit www.myana.org or @TheNewANA1

Chat now!
6.23282