Lewy body dementia
Lewy body dementia (LBD, sometimes referred to as Lewy body disorder) is an umbrella term that includes Parkinson's disease dementia (PDD) and dementia with Lewy bodies (DLB), two dementias characterized by abnormal deposits of the protein alpha-synuclein in the brain.
Signs and symptoms
The synucleinopathies (DLB, PDD and Parkinson's disease) have shared features of parkinsonism, impaired cognition, sleep disorders, and visual hallucinations. A core feature is REM sleep behavior disorder (RBD), in which individuals lose normal muscle paralysis during REM sleep, and act out their dreams. Other frequent symptoms include visual hallucinations; marked fluctuations in attention or alertness; and slowness of movement, trouble walking, or rigidity. The autonomic nervous system is usually affected, and can result in symptoms like changes in blood pressure, heart and gastrointestinal function, and constipation. Mood changes such as depression and apathy are common.
REM sleep behavior disorder may appear decades before any other symptoms. On autopsy, 94 to 98% of individuals with polysomnography-confirmed RBD are found to have a synucleinopathy—most commonly DLB or Parkinson's disease, in about equal proportions. Other symptoms of the specific synucleinopathy usually manifest within 15 years of the diagnosis of RBD, but may emerge up to 50 years after RBD diagnosis.
Dementia with Lewy bodies is distinguished from Parkinson's disease dementia by the time frame in which dementia symptoms appear relative to parkinsonian symptoms. DLB is diagnosed when cognitive symptoms begin before or at the same time as parkinsonism, while PDD is the diagnosis when Parkinson's disease is well established before the dementia occurs.
Dementia with Lewy bodies and Parkinson's disease dementia are similar in many ways, suggesting there may be a common pathophysiological mechanism, with PDD and DLB at opposite ends of a LBD spectrum, and a shared component of protein deposits in Lewy bodies and Lewy neurites. Despite differences in the timing of the appearance of symptoms, the two dementias "show remarkably convergent neuropathological changes at autopsy".
Society and culture
Advocacy and awareness
Lewy body dementias are more often misdiagnosed than any other common dementia. Most people with DLB had not heard of the condition prior to diagnosis; general awareness about LBD lags well behind that of Parkinson's and Alzheimer's diseases, even though LBD is the second most common dementia, after Alzheimer's. It is not only frustrating for families and caregivers to find that few people, including many healthcare professionals, are knowledgeable about LBD; lack of knowledge can have significant health consequences because people with LBD have severe sensitivity to antipsychotics often used to treat the symptoms. The Lewy Body Dementia Association (LBDA) and the Lewy Body Society promote awareness and provide support that helps society, by reducing costly use of healthcare, and families with LBD, by reducing stress. These organization, and others in Argentina, Australia and Japan, help raise knowledge and help families with LBD become advocates to raise awareness about the disease.
The British author and poet Mervyn Peake died in 1968 and was diagnosed posthumously as a probable case of DLB in a 2003 paper published in JAMA Neurology. Sahlas said his death was "variously ascribed to Alzheimer disease, Parkinson disease, or postencephalitic parkinsonism". Based on signs in his work and letters of progressive deterioration, fluctuating cognitive decline, deterioration in visuospatial function, declining attention span, and visual hallucinations and delusions, his may be the earliest known case where DLB was found to have been the likely cause of death.
Robin Williams, the American actor and comedian, died by suicide on August 11, 2014. He had been diagnosed with Parkinson's disease, and according to his widow he experienced depression, anxiety and increasing paranoia. Upon autopsy his widow said he was found to have diffuse DLB. Ian G. McKeith, a dementia researcher and professor of old age psychiatry, commented that DLB was still too little known, and that Williams' symptoms were explained by DLB. The LBDA clarified the distinction between the term used in the autopsy report, diffuse Lewy body dementia, which is more commonly called diffuse Lewy body disease and refers to the underlying disease process; and the umbrella term, Lewy body dementia, which encompasses both PDD and DLB. According to Dennis Dickson, the LBDA spokesperson, "The report confirms he experienced depression, anxiety and paranoia, which may occur in either Parkinson's disease or dementia with Lewy bodies", adding that, in Parkinson's, "Lewy bodies are generally limited in distribution, but in DLB, the Lewy bodies are spread widely throughout the brain, as was the case with Robin Williams."
Other entertainers and artists who had or died from LBD include Estelle Getty, an actress known for her role in the television series The Golden Girls, Nicholas King, a US actor and horticulturist, actress Dina Merrill, Donald Featherstone, who created the plastic pink flamingo, American radio and television host Casey Kasem, and Canadian singer Pierre Lalonde.
Individuals from industry or government who had or died from LBD are Seymour Berry, US Director of the Bureau of Engraving and Printing, Los Angeles Times publisher Otis Chandler, and Philip J. Rock, a US Democratic politician of the Illinois Senate.
In the sports realm, Stan Mikita, Canadian ice hockey player, and Jerry Sloan, American professional basketball player and coach, have been diagnosed with DLB. Andy Carey was a US Major League Baseball player for the New York Yankees who died from LBD.
Sleepwalk with Me is a book, a one-man comedy and a film about a young man with relationship problems and REM sleep behavior disorder. Hand of God, the Amazon Prime series, deals with a judge who claims to have the this type of dementia.
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