Medicine:Lewy body dementia

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Short description: An umbrella term covering two types of dementia

Lewy body dementia (LBD, or Lewy body disorder) is an umbrella term[1] that encompasses two similar dementias, both of which are characterized by abnormal deposits of the protein alpha-synuclein in the brain:

The two conditions have similar features, may have similar causes, and can be viewed as part of a spectrum. As of 2014, they were more often misdiagnosed than any other common dementia.

Classification

The synucleinopathies (DLB, PDD, and Parkinson's disease) are characterized by shared features of parkinsonism motor symptoms, neuropsychiatric symptoms, impaired cognition, sleep disorders, and visual hallucinations.[5][6] The Lewy body dementias are considered to consist of the two related disorders, dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD) that are distinguished by the timing when cognitive and motor symptoms appear.[7]

Cause and mechanisms

Further information: Dementia with Lewy bodies § Pathophysiology

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.[8] Lewy bodies and neurites have been found to develop from aggregation of misfolded alpha-synuclein, a protein thought to assist in neurotransmitter release and vesicle turnover; whether these misfolded proteins are responsible for the neurodegenerative effects remains unclear, and no definitive link between Lewy bodies and neurodegenerative effects has been found.[9] 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 DLB is unclear as of 2020, but there is likely to be genetic overlap, and the two conditions may represent different points on a continuum.[10]

Diagnosis

Further information: Dementia with Lewy bodies § Diagnosis

Dementia with Lewy bodies and Parkinson's disease dementia have similar neuropathological features, but these features are highly variable and the conditions should not be distinguished on pathological features alone.[9] Generally, DLB is distinguished from PDD 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.[6]

Epidemiology

As of 2014, the Lewy body dementias affect about 1.3 million people in the US and 140,000 in the UK.[11] LBD usually develops after the age of 50.[12] Between 5% and 25% of diagnosed dementias in older adults are due to Lewy body dementias.[7]

Society and culture

Advocacy and awareness

As of 2014, the Lewy body dementias were more often misdiagnosed than any other common dementia.[11] 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.[11] 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.[11] 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.[11] 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.[11]

Notable individuals

His widow said Robin Williams (shown in 2011) was diagnosed on autopsy with Lewy bodies.[13][14]

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.[15] Sahlas said his death was "variously ascribed to Alzheimer disease, Parkinson disease, or postencephalitic parkinsonism".[15] 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.[15]

Robin Williams, the American actor and comedian, committed suicide on August 11, 2014. He had been diagnosed with Parkinson's disease,[13] and according to his widow, he experienced depression, anxiety, and increasing paranoia.[14] Upon autopsy, his widow said he was found to have diffuse DLB.[16] 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.[17] 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.[18] 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."[18]

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,[19] Nicholas King, a US actor and horticulturist,[20] actress Dina Merrill,[21] Donald Featherstone, who created the plastic pink flamingo,[22] American radio and television host Casey Kasem,[23] and Canadian singer Pierre Lalonde.[24][25]

Individuals from industry or government who had or died from LBD are Seymour Berry, US Director of the Bureau of Engraving and Printing,[26] Los Angeles Times publisher Otis Chandler,[27] Philip J. Rock, a US Democratic politician of the Illinois Senate,[28] and U.S. media mogul and philanthropist Ted Turner.[29]

Arnold R. Hirsch, an American historian who taught at the University of New Orleans,[30] and Jessie Isabelle Price, an American veterinary microbiologist,[31] died from LBD.

In the sports realm, Jerry Sloan, American professional basketball player and coach, died from DLB.[32] Major League Baseball players Andy Carey,[33] and Bill Buckner died of LBD.[34] Stan Mikita, Canadian ice hockey player, was diagnosed with possible DLB,[35] but a post-mortem brain autopsy found that he had chronic traumatic encephalopathy.[36]

In popular culture

  • Hand of God, the Amazon Prime series, deals with a judge who claims to have this type of dementia.
  • Sleepwalk with Me is a book, one-man comedy, and film about a young man with relationship problems and RBD, a precursor to synucleinopathy, including LBD.[37]

References

  1. ↑ "Lewy body dementias". Lancet 386 (10004): 1683–97. October 2015. doi:10.1016/S0140-6736(15)00462-6. PMID 26595642. 
  2. ↑ 2.0 2.1 "Lewy Body Dementias: Dementia With Lewy Bodies and Parkinson Disease Dementia". Continuum (Minneap Minn) 22 (2 Dementia): 435–63. April 2016. doi:10.1212/CON.0000000000000309. PMID 27042903. 
  3. ↑ "Structural and Functional Neuroimaging of Visual Hallucinations in Lewy Body Disease: A Systematic Literature Review". Brain Sci 7 (7): 84. July 2017. doi:10.3390/brainsci7070084. PMID 28714891. 
  4. ↑ "Lewy Body Disorders". Neurol Clin 35 (2): 325–338. May 2017. doi:10.1016/j.ncl.2017.01.004. PMID 28410662. 
  5. ↑ "New Therapeutic Strategies for Lewy Body Dementias". Curr Neurol Neurosci Rep 17 (9): 68. September 2017. doi:10.1007/s11910-017-0778-2. PMID 28741230. 
  6. ↑ 6.0 6.1 "Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium". Neurology 89 (1): 88–100. July 2017. doi:10.1212/WNL.0000000000004058. PMID 28592453. 
  7. ↑ 7.0 7.1 "Non-pharmacological interventions for Lewy body dementia: a systematic review". Psychol Med 48 (11): 1749–1758. August 2018. doi:10.1017/S0033291717003257. PMID 29143692. 
  8. ↑ "Current concepts and controversies in the pathogenesis of Parkinson's disease dementia and Dementia with Lewy Bodies". F1000Res 6: 1604. 2017. doi:10.12688/f1000research.11725.1. PMID 28928962. 
  9. ↑ 9.0 9.1 Latimer CS, Montine TJ. "Epidemiology, pathology, and pathogenesis of dementia with Lewy bodies". UpToDate, Inc.. https://www.uptodate.com/contents/epidemiology-pathology-and-pathogenesis-of-dementia-with-lewy-bodies. 
  10. ↑ "New evidence on the management of Lewy body dementia". Lancet Neurol 19 (2): 157–69. February 2020. doi:10.1016/S1474-4422(19)30153-X. PMID 31519472. https://ore.exeter.ac.uk/repository/bitstream/handle/10871/36535/Management%20Lewy%20body%20dementia_versionsubmittedtoTLNwithappendix.pdf?sequence=10&isAllowed=y. 
  11. ↑ 11.0 11.1 11.2 11.3 11.4 11.5 "Advocacy, education, and the role of not-for-profit organizations in Lewy body dementias". Alzheimers Res Ther 6 (5): 59. 2014. doi:10.1186/s13195-014-0059-0. PMID 26082807. 
  12. ↑ "Lewy body dementia: Hope through research". US National Institutes of Health. December 8, 2017. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Hope-Through-Research/Lewy-Body-Dementia-Hope-Through-Research. Retrieved April 6, 2018. 
  13. ↑ 13.0 13.1 Gallman S (November 4, 2015). "Robin Williams' widow speaks: Depression didn't kill my husband". CNN. Archived from the original on November 4, 2015. https://web.archive.org/web/20151104002632/http://www.cnn.com/2015/11/03/health/robin-williams-widow-susan-williams/index.html. Retrieved April 6, 2018. 
  14. ↑ 14.0 14.1 "The terrorist inside my husband's brain". Neurology 87 (13): 1308–11. September 2016. doi:10.1212/WNL.0000000000003162. PMID 27672165. 
  15. ↑ 15.0 15.1 15.2 "Dementia with Lewy bodies and the neurobehavioral decline of Mervyn Peake". Arch. Neurol. 60 (6): 889–92. June 2003. doi:10.1001/archneur.60.6.889. PMID 12810496. 
  16. ↑ Robbins R (September 30, 2016). "How Lewy body dementia gripped Robin Williams". Scientific American. https://www.scientificamerican.com/article/how-lewy-body-dementia-gripped-robin-williams1/. Retrieved April 9, 2018. 
  17. ↑ McKeith, IG. "Robin Williams had dementia with Lewy bodies -- so, what is it and why has it been eclipsed by Alzheimer's?". The Conversation. Archived from the original on November 4, 2016. https://web.archive.org/web/20161104010452/https://theconversation.com/robin-williams-had-dementia-with-lewy-bodies-so-what-is-it-and-why-has-it-been-eclipsed-by-alzheimers-50221. 
  18. ↑ 18.0 18.1 "LBDA Clarifies Autopsy Report on Comedian, Robin Williams". Lewy Body Dementia Association. November 10, 2014. https://www.lbda.org/content/lbda-clarifies-autopsy-report-comedian-robin-williams. Retrieved April 19, 2018. 
  19. ↑ Carlson M (July 24, 2008). "Obituary: Estelle Getty". theguardian.com. Archived from the original on September 2, 2013. https://web.archive.org/web/20130902115947/http://www.theguardian.com/culture/2008/jul/24/television.television. Retrieved October 13, 2013. 
  20. ↑ McLellan D (April 23, 2012). "Nicholas King dies at 79; actor helped preserve the Watts Towers". The Los Angeles Times. http://www.latimes.com/news/obituaries/la-me-nicholas-king-20120423,0,3790852.story. Retrieved April 19, 2018. 
  21. ↑ Dangremond S (May 23, 2017). "Actress and philanthropist Dina Merrill dies at 93". Town and Country Magazine. https://www.townandcountrymag.com/society/a9914626/dina-merrill-obituary/. Retrieved March 22, 2018. 
  22. ↑ Woo E (June 24, 2015). "Don Featherstone dies at 79; creator of the plastic pink flamingo". Los Angeles Times. http://www.latimes.com/local/obituaries/la-me-0624-donald-featherstone-20150624-story.html. Retrieved March 22, 2018. 
  23. ↑ Caffrey J (April 18, 2016). "Casey Kasem and a lesson about end-of-life care". CNN. https://www.cnn.com/2014/06/20/health/casey-kasem-end-of-life-care/index.html. Retrieved March 22, 2018. 
  24. ↑ Papineau P (June 23, 2016). "L'idole d'une génération s'éteint" (in fr). Le Devoir. https://www.ledevoir.com/culture/actualites-culturelles/474105/deces-de-pierre-lalonde. Retrieved April 9, 2018. 
  25. ↑ Belanger C (June 22, 2016). "Pierre Lalonde souffrait aussi de la démence à corps de Lewy" (in fr). Le Journal de Montréal. http://www.journaldemontreal.com/2016/06/22/pierre-lalonde-souffrait-aussi-de-la-demence-de-corps-de-lewy/. Retrieved March 22, 2018. 
  26. ↑ "Seymour Berry, 86; Headed U.S. Agency". Washington Post. December 27, 2008. https://www.washingtonpost.com/wp-dyn/content/article/2008/12/26/AR2008122601714.html. Retrieved April 19, 2018. 
  27. ↑ Shaw D, Landsberg M (February 27, 2006). "L.A. icon Otis Chandler dies at 78". The Los Angeles Times. Archived from the original on April 26, 2007. https://web.archive.org/web/20070426114430/http://www.latimes.com/news/local/la-me-chandler-obit,0,7195252.story?page=1&coll=la-home-headlines. Retrieved July 23, 2008. 
  28. ↑ "Philip Rock, ex-Senate leader known for mentoring and bipartisanship, dies". Chicago Sun-Times. January 29, 2016. Archived from the original on February 1, 2016. https://web.archive.org/web/20160201093023/http://chicago.suntimes.com/news/7/71/1288547/philip-rock-longest-serving-illinois-senate-president-dies. Retrieved April 19, 2018. 
  29. ↑ Deerwester J (September 28, 2018). "Ted Turner has Lewy Body Dementia". USA TODAY. https://www.usatoday.com/story/life/people/2018/09/28/ted-turner-has-lewy-body-dementia-what-neurological-disorder/1456677002/. Retrieved September 30, 2018. 
  30. ↑ O'Donnell M (March 26, 2018). "Arnold R. Hirsch dies; analyzed Chicago segregation in influential book". Chicago Sun Times. https://chicago.suntimes.com/news/arnold-r-hirsch-dies-analyzed-chicago-segregation-in-influential-book/. Retrieved April 19, 2018. 
  31. ↑ "Jessie Isabelle Price Dies On November 12". The Southampton Press. November 23, 2015. http://www.27east.com/news/article.cfm/Quogue/131382/Jessie-Isabelle-Price-Dies-On-November-12. Retrieved April 19, 2018. 
  32. ↑ "Hall of Fame coach Jerry Sloan passes away at 78". National Basketball Association (Press release). NBA Media Ventures, LLC. May 22, 2020. Retrieved June 8, 2020.
  33. ↑ Weber B (January 7, 2012). "Andy Carey, Third Baseman for 1950s Yankees, Dies at 80". https://www.nytimes.com/2012/01/08/sports/baseball/andy-carey-third-baseman-for-1950s-yankees-dies-at-80.html. Retrieved April 19, 2018. 
  34. ↑ Kelly, Matt (May 27, 2019). "Batting champ, All-Star Buckner dies at 69". https://www.mlb.com/news/bill-buckner-dies. Retrieved May 27, 2019. 
  35. ↑ Kuc C (June 15, 2015). "For Stan Mikita, all the Blackhawks memories are gone". Chicago Tribune. Archived from the original on June 16, 2015. https://web.archive.org/web/20150616044959/http://www.chicagotribune.com/sports/hockey/blackhawks/ct-stan-mikita-blackhawks-spt-0615-20150614-story.html#page=1. Retrieved March 22, 2018. 
  36. ↑ "Study shows hockey Hall of Famer Stan Mikita suffered from CTE". USA Today (Associated Press). September 13, 2019. https://www.usatoday.com/story/sports/nhl/2019/09/13/study-shows-hockey-great-stan-mikita-suffered-from-cte/40145889/. Retrieved June 8, 2020. 
  37. ↑ "Sleepwalk with Me: Comedian's sleep disorder experience comes to film". American Academy of Sleep Medicine. January 26, 2012. http://www.sleepeducation.org/news/2012/01/27/sleepwalk-with-me-comedian's-sleep-disorder-experience-comes-to-film. Retrieved April 22, 2018. 

External links

Classification




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