“Robin’s Wish” is a film to be released today that chronicles comedian and actor Robin Williams’ experiences with and symptoms of Lewy Body Dementia (LBD). The film describes the disease, how it affected his personal and professional life, and current research efforts to understand and treat this fairly common cause of dementia. This documentary is the product of his wife Susan Schneider Williams’ passion to educate people about LBD so that other families do not have to go through the heart-wrenching experiences she and Robin endured.
In the last year of his life, Robin Williams experienced a puzzling pattern of behavior. His symptoms included personality changes, confusion, forgetfulness, anxiety, paranoia, hallucinations, and problems with movement. Friends, family, and film colleagues recognized that he was not himself. He too felt that something was wrong, but he did not know what or why. Robin and his wife, Susan, sought help from a variety of medical specialists but there were no definitive answers. It was not until after his death that an autopsy demonstrated advanced stages of a brain disease called Lewy Body Dementia (LBD). This diagnosis led Susan Schneider Williams on an odyssey to learn how LBD explained Robin’s symptoms and to raise awareness of the disease. LBD is a less well-known form of dementia that affects approximately 1 million Americans. There is considerable overlap between LBD and the cognitive decline that occurs in many individuals with longstanding Parkinson’s disease.
Lewy bodies are clumps of proteins that form round deposits inside nerve cells. They are found throughout the brain in LBD, Multiple System Atrophy, and Parkinson’s disease-related dementia. In early Parkinson’s disease, Lewy Bodies tend to be restricted to dopamine cells in the midbrain, but in later stages of Parkinson’s with dementia, Lewy Bodies are also found in higher brain circuits. In contrast, in Dementia with Lewy Bodies, which was Robin’s technical diagnosis, the Lewy Bodies occur in the higher brain circuits first, and signs of Parkinson’s may or may not come later.
Lewy Bodies are primarily composed of a protein called alpha synuclein. Alpha synuclein is important in the communication of signals between nerve cells. However, when synuclein aggregates and forms clumps inside the cells it is likely toxic, and can spread throughout the brain, damaging neurons along the way. This damage can lead to the devastating symptoms of Parkinson’s or LBD. Parkinson’s has classic diagnostic signs like tremor, rigidity and slowness of movement. However, Dementia with Lewy Bodies doesn’t have these telltale signs, but instead presents primarily with cognitive and behavioral disturbances such as those experienced by Robin and described in the film. Importantly, Dementia with Lewy Bodies is notoriously difficult to diagnose: changes in cognition and hallucinations are often clues, but a definitive diagnosis can only be made by brain examination after death. Researchers are working to develop tests to detect aggregates of synuclein – a promising avenue for future diagnosis. There is currently no cure for Dementia with Lewy Bodies, although therapeutics are available to help manage symptoms. There are a number of potential treatments for synuclein diseases that are in different stages of development and clinical trials.
Since Robin’s death, Susan has immersed herself in becoming an advocate for LBD awareness. When the film was in production, Susan came to the NIH to interview me, NIH lab chiefs, and program directors. Many of Robin’s friends, and Susan herself, suspected that her husband was suffering from a severe psychiatric disorder such as depression or schizophrenia. We discussed the artificial nature of the distinction between neurological and severe psychiatric disorders. They are all consequences of disorders of the brain’s circuitry. When there is a miswiring in the brain for any reason, pathologic behavior or loss of cognitive abilities can result. Susan’s years of work have paid off; the theatrical release of “Robin’s Wish” will start streaming on September 1st on Video on Demand services. I found it deeply moving, and I highly recommend it. Additionally, our friends at the Lewy Body Dementia Association are producing a medical/scientific version of the documentary for the healthcare community. They feel strongly that the research community could benefit from learning more about LBD, and we agree. This second version of the film will be featured in a launch party on October 29th, and I will serve as a panelist after the airing.
The release of “Robin’s Wish” highlights the need to understand, treat, and even prevent LBD; this is an active and ongoing area of research at NIH. Through NIH support of basic, translational, and clinical research, LBD researchers are investigating a variety of aspects of the disease including genetics, learning how brain cells are affected, and environmental factors that may influence of developing the disease. In FY19, NIH spent $66 million on Lewy Body Dementia research. NIH has also created the Neurobiobank, a national resource for investigators utilizing human post-mortem brain tissue and related biospecimens for their research to understand conditions of the nervous system. At NINDS, investments in research on biomarkers for the Lewy Body Dementias program aim to identify signals specific to the disease, thereby facilitating disease diagnosis and tracking efficacy of potential treatments. I have also written previously about a suite of forthcoming funding opportunities related to Alzheimer’s Disease and Related Dementias, which includes LBD. Current funding opportunities now seek to understand mechanisms of vulnerability in LBD, as well as mechanisms of pathological spread of abnormal proteins in LBD. More information and additional funding opportunities can be found at the NINDS “Focus On Lewy Body Dementia” page.
At NINDS, our mission is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease. In achieving this goal, we also recognize the critical importance of raising awareness of diseases to help individuals and their families who may be affected by these devastating disorders.
Robin Williams brought so much joy to so many people through his acting and his charity work, and there is no doubt that he will always be cherished for his brilliant performances on screen and stage. “Robin’s Wish” was not a role he auditioned for or sought out, but Susan’s work through this documentary honors his wish of helping other people to not be afraid (or ‘to be fearless’). We hope that NINDS can play a supporting role in making this wish come true.