Lewy body dementia (LBD) is two similar and common subtypes ofdementia:^[1]dementia with Lewy bodies (DLB) andParkinson's disease dementia (PDD).^[2][3][4][5] Both are characterized by changes in thinking, movement, behavior, and mood.^[1] The two conditions have similar features and may have similar causes, and are believed to belong on a spectrum ofLewy body disease^[2] that includesParkinson's disease.^[5] As of 2014, they were more often misdiagnosed than any other common dementia.^[6]

The exact cause is unknown, but involves widespread deposits of abnormal clumps of protein that form inneurons of the diseased brain.^[7] Known asLewy bodies (discovered in 1912 byFrederic Lewy^[8]) andLewy neurites, these clumps affect both thecentral nervous system and theautonomic nervous system.^[9] The fifth revision of theDiagnostic and Statistical Manual of Mental Disorders (DSM-5) gives Lewy body disease as the causative subtype of dementia with Lewy bodies, andParkinson's disease as the causative subtype of Parkinson's disease dementia.^[10] Dementia with Lewy bodies is marked by the presence of Lewy bodies primarily in thecortical regions, and Parkinson's disease dementia with Lewy bodies primarily in the subcorticalbasal ganglia.^[11]

Thesynucleinopathies (dementia with Lewy bodies,Parkinson's disease dementia, andParkinson's disease) are characterized by shared features ofparkinsonism motor symptoms, neuropsychiatric symptoms, impaired cognition,sleep disorders, andvisual hallucinations.^[12][13] The Lewy body dementias—dementia with Lewy bodies (DLB), and Parkinson's disease dementia (PDD)—are distinguished by the timing when cognitive and motor symptoms appear.^[14] The two Lewy body dementias are often considered to belong on a spectrum of Lewy body disease that includes Parkinson's disease.^[2][5]

MeSH lists Lewy body disease in several categories: as a nervous system disease in two listings, one as a basal ganglia Parkinsonian movement disorder and the other under brain disease as a dementia; as a neurodegenerative disorder listed as a synucleinopathy; and as a neurocognitive disorder listed with dementia.^[15]

A genetic architecture that predisposes an individual to some diseasephenotypes is found in Parkinson's disease and the Lewy body dementias. The presence of Lewy bodies is a link between these disorders; the term "diseases with Lewy bodies" therefore may be more accurate than "Lewy body disease".^[16]

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 Lewy body disease spectrum,^[2] and a shared component of protein deposits inLewy bodies andLewy neurites.^[17] Lewy bodies and neurites have been found to develop from the aggregation of misfoldedalpha-synuclein, a protein thought to assist in neurotransmitter release and vesicle turnover; whether thesemisfolded proteins are responsible for the neurodegenerative effects remains unclear, and no definitive link between Lewy bodies and neurodegenerative effects has been found.^[18] DSM-5 gives Lewy body disease as the causative subtype of DLB, andParkinson's disease as the causative subtype of PDD.^[10] DLB is marked by the presence of Lewy bodies primarily in thecortical regions, and PDD with Lewy bodies primarily in the subcorticalbasal ganglia.^[11]

Despite differences in the timing of the appearance of symptoms, the two dementias "show remarkably convergent neuropathological changes at autopsy".^[2] The relationship between Parkinson's disease dementia and dementia with Lewy bodies is unclear as of 2020, but there is likely to be genetic overlap, and the two conditions may represent different points on a continuum.^[19]

Dementia with Lewy bodies and Parkinson's disease dementia have similar neuropathological features, but these features are highly variable and the conditions cannot be distinguished on pathological features alone.^[18] Generally, dementia with Lewy bodies is distinguished from Parkinson's disease dementia by the time frame in which dementia symptoms appear relative to parkinsonian symptoms and is diagnosed when cognitive symptoms begin before or at the same time asparkinsonism. Parkinson's disease dementia is the diagnosis when Parkinson's disease is already well established before the dementia occurs.^[13]

Between 5% and 25% of diagnosed dementias in older adults are due to one of the Lewy body dementias.^[14][a] As of 2014, the Lewy body dementias affect about 1.3 million people in the US and 140,000 in the UK.^[6]

LBD usually develops after the age of 50.^[8] Men are more likely to be diagnosed than women.^[8]

Life expectancy of people with one of the LBD is reduced; following diagnosis it ranges on average from five to eight years.^[22]

As of 2014, the Lewy body dementias were more often misdiagnosed than any other common dementia.^[6] Most people with DLB had not heard of the condition prior to diagnosis; general awareness about LBD lags well behind that ofParkinson's andAlzheimer's diseases, even though LBD is the second most common dementia, after Alzheimer's.^[6] It is not only frustrating for families andcaregivers 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 toantipsychotics often used to treat the symptoms.^[6] TheLewy Body Dementia Association (LBDA) and the UKLewy Body Society promote awareness and provide support that helps society, by reducing costly use of healthcare, and families with LBD, by reducing stress.^[6] These organizations, and others in Argentina, Australia and Japan, help raise knowledge and help families with LBD become advocates to raise awareness about the disease.^[6]

Robin Williams, the American actor and comedian, died on August 11, 2014. Before his suicide, he had been diagnosed with Parkinson's disease,^[23] and according to his widow, Susan Schneider Williams, he experienced depression,anxiety, and increasingparanoia.^[24] Upon autopsy, his widow said he was found to have diffuse Lewy body disease,^[23][24][25] while the autopsy used the termdiffuse Lewy body dementia.^[26] The vocabulary associated with Lewy pathology causes confusion.^[27]Lewy body dementia (the umbrella term that encompasses the clinical diagnoses of dementia with Lewy bodies and Parkinson's disease dementia) differs fromLewy body disease (the term used to describe pathological findings of Lewy bodies on autopsy).^[27] Dennis Dickson, a spokesperson for the Lewy Body Dementia Association, clarified the distinction by stating thatdiffuse Lewy body dementia is more commonly calleddiffuse Lewy body disease and refers to the underlying disease process.^[26] According to Dickson, "Lewy bodies are generally limited in distribution" in early Parkinson's disease, while indementia with Lewy bodies, "the Lewy bodies are spread widely throughout the brain, as was the case with Robin Williams."^[26]Ian G. McKeith, professor and researcher of Lewy body dementias, commented that Williams' symptoms and autopsy findings were explained by dementia with Lewy bodies.^[28]

The British author and poetMervyn Peake died in 1968 and was diagnosedposthumously as a probable case of DLB in a 2003 paper published inJAMA Neurology.^[29] Sahlas said his death was "variously ascribed to Alzheimer disease, Parkinson disease, or postencephalitic parkinsonism".^[29] Based on signs in his work and letters of progressive deterioration, fluctuating cognitive decline, deterioration in visuospatial function, decliningattention 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.^[29]

Other entertainers and artists who have or died from LBD includeEstelle Getty, the actress known for her role as Sophia on the television seriesThe Golden Girls,^[30]Nicholas King, a US actor and horticulturist,^[31] actressDina Merrill,^[32]Donald Featherstone, who created the plastic pink flamingo,^[33] American radio and television hostCasey Kasem,^[34] Canadian singerPierre Lalonde,^[35][36] graphic artist/film set designerRon Cobb,^[37] American actorFrank Bonner,^[38] Canadian musician and actorAndré Gagnon,^[39] andMark Volman, founding member of the bandthe Turtles.^[40]

British authorMichael Bywater died of LBD.^[41]

Individuals from industry or government who have or died from LBD includeSeymour Berry, USDirector of the Bureau of Engraving and Printing,^[42]Los Angeles Times publisherOtis Chandler,^[43]Philip J. Rock,a USDemocratic politician of theIllinois Senate,^[44] U.S. media mogul and philanthropistTed Turner,^[45] and Indian-born British billionaireS. P. Hinduja.^[46]

Arnold R. Hirsch, an American historian who taught at theUniversity of New Orleans,^[47] andJessie Isabelle Price, an American veterinarymicrobiologist,^[48] died from LBD.

In the sports realm,Jerry Sloan, American professional basketball player and coach, died from LBD.^[49]Major League Baseball playersTom Seaver,^[50]Andy Carey,^[51] andBill Buckner died of LBD.^[52]Stan Mikita, Canadian ice hockey player, was diagnosed with possible LBD,^[53] but a post-mortem brain autopsy found that he hadchronic traumatic encephalopathy.^[54]

Robin's Wish, a documentary exploring Robin Williams's Lewy body disease and how it contributed to his death, was released in September 2020.^[55][56][57]

Sleepwalk with Me is a book, one-man comedy, and film about a young man with relationship problems andRBD, a precursor tosynucleinopathy, including LBD.^[58]

• Lewy body dementia
• Cognitive disorders
• Dementia

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