Lewy body dementia

Lewy body dementia (LBD, sometimes referred to as Lewy body disorder) is an umbrella term[1] 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.[2][3][4]

Signs and symptoms

The synucleinopathies (DLB, PDD and Parkinson's disease) have shared features of parkinsonism, impaired cognition, sleep disorders, and visual hallucinations.[3] A core feature is REM sleep behavior disorder (RBD), in which individuals lose normal muscle paralysis during REM sleep, and act out their dreams.[5][6] Other frequent symptoms include visual hallucinations; marked fluctuations in attention or alertness; and slowness of movement, trouble walking, or rigidity.[5] The autonomic nervous system is usually affected, and can result in symptoms like changes in blood pressure, heart and gastrointestinal function, and constipation.[7] Mood changes such as depression and apathy are common.[5]

REM sleep behavior disorder may appear decades before any other symptoms.[6] On autopsy, 94 to 98% of individuals with polysomnography-confirmed RBD are found to have a synucleinopathy—most commonly DLB or Parkinson's disease,[8][9][10] in about equal proportions.[11] Other symptoms of the specific synucleinopathy usually manifest within 15 years of the diagnosis of RBD,[1] but may emerge up to 50 years after RBD diagnosis.[8]

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.[5]

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.[12] Despite differences in the timing of the appearance of symptoms, the two dementias "show remarkably convergent neuropathological changes at autopsy".[2]

Epidemiology

The Lewy body dementias affect about 1.3 million people in the US and 140,000 in the UK.[13] LBD usually develops after the age of 50.[14]

Society and culture

Advocacy and awareness

Lewy body dementias are more often misdiagnosed than any other common dementia.[13] 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.[13] 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.[13] 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.[13] 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.[13]

Notable individuals

His widow said Robin Williams (shown in 2011) was diagnosed on autopsy with Lewy bodies.[15][16]

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.[17] Sahlas said his death was "variously ascribed to Alzheimer disease, Parkinson disease, or postencephalitic parkinsonism".[17] 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.[17]

Robin Williams, the American actor and comedian, died by suicide on August 11, 2014. He had been diagnosed with Parkinson's disease,[15] and according to his widow he experienced depression, anxiety and increasing paranoia.[16] Upon autopsy his widow said he was found to have diffuse DLB.[18] 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.[19] 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.[20] 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."[20]

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,[21] Nicholas King, a US actor and horticulturist,[22] actress Dina Merrill,[23] Donald Featherstone, who created the plastic pink flamingo,[24] American radio and television host Casey Kasem,[25] and Canadian singer Pierre Lalonde.[26][27]

Individuals from industry or government who had or died from LBD are Seymour Berry, US Director of the Bureau of Engraving and Printing,[28] Los Angeles Times publisher Otis Chandler,[29] Philip J. Rock, a US Democratic politician of the Illinois Senate.[30] and U.S. media mogul and philanthropist Ted Turner.[31]

Arnold R. Hirsch, a US historian who taught at the University of New Orleans,[32] and Jessie Isabelle Price a US veterinary microbiologist[33] died from LBD.

In the sports realm, Stan Mikita, Canadian ice hockey player,[34] and Jerry Sloan, American professional basketball player and coach,[35] have been diagnosed with DLB. Andy Carey was a US Major League Baseball player for the New York Yankees who died from LBD.[36]

In film

Hand of God, the Amazon Prime series, deals with a judge who claims to have the this type of dementia.

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, a precursor to synucleinopathy, including LBD.[37]

References

  1. 1 2 Walker Z, Possin KL, Boeve BF, Aarsland D (October 2015). "Lewy body dementias". Lancet (Review). 386 (10004): 1683–97. doi:10.1016/S0140-6736(15)00462-6. PMC 5792067. PMID 26595642.
  2. 1 2 Gomperts SN (April 2016). "Lewy Body Dementias: Dementia With Lewy Bodies and Parkinson Disease Dementia". Continuum (Minneap Minn). 22 (2 Dementia): 435–63. doi:10.1212/CON.0000000000000309. PMC 5390937. PMID 27042903.
  3. 1 2 Pezzoli S, Cagnin A, Bandmann O, Venneri A (July 2017). "Structural and Functional Neuroimaging of Visual Hallucinations in Lewy Body Disease: A Systematic Literature Review". Brain Sci. 7 (7). doi:10.3390/brainsci7070084. PMC 5532597. PMID 28714891.
  4. ↑ Galasko D (May 2017). "Lewy Body Disorders". Neurol Clin. 35 (2): 325–338. doi:10.1016/j.ncl.2017.01.004. PMID 28410662.
  5. 1 2 3 4 McKeith IG, Boeve BF, Dickson DW, et al. (July 2017). "Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium". Neurology (Review). 89 (1): 88–100. doi:10.1212/WNL.0000000000004058. PMC 5496518. PMID 28592453.
  6. 1 2 St Louis EK, Boeve AR, Boeve BF (May 2017). "REM sleep behavior disorder in Parkinson's disease and other synucleinopathies". Mov. Disord. (Review). 32 (5): 645–58. doi:10.1002/mds.27018. PMID 28513079.
  7. ↑ Palma JA, Kaufmann H (March 2018). "Treatment of autonomic dysfunction in Parkinson disease and other synucleinopathies". Mov. Disord. (Review). 33 (3): 372–90. doi:10.1002/mds.27344. PMID 29508455.
  8. 1 2 St Louis EK, Boeve BF (November 2017). "REM sleep behavior disorder: Diagnosis, clinical implications, and future directions". Mayo Clin. Proc. (Review). 92 (11): 1723–36. doi:10.1016/j.mayocp.2017.09.007. PMID 29101940.
  9. ↑ Boot BP, McDade EM, McGinnis SM, Boeve BF (December 2013). "Treatment of dementia with Lewy bodies". Curr Treat Options Neurol (Review). 15 (6): 738–64. doi:10.1007/s11940-013-0261-6. PMC 3913181. PMID 24222315.
  10. ↑ Boot BP (2015). "Comprehensive treatment of dementia with Lewy bodies". Alzheimers Res Ther (Review). 7 (1): 45. doi:10.1186/s13195-015-0128-z. PMC 4448151. PMID 26029267.
  11. ↑ Arnaldi D, Antelmi E, St Louis EK, Postuma RB, Arnulf I (December 2017). "Idiopathic REM sleep behavior disorder and neurodegenerative risk: To tell or not to tell to the patient? How to minimize the risk?". Sleep Med Rev (Review). 36: 82–95. doi:10.1016/j.smrv.2016.11.002. PMID 28082168.
  12. ↑ Weil RS, Lashley TL, Bras J, Schrag AE, Schott JM (2017). "Current concepts and controversies in the pathogenesis of Parkinson's disease dementia and Dementia with Lewy Bodies". F1000Res (Review). 6: 1604. doi:10.12688/f1000research.11725.1. PMC 5580419. PMID 28928962.
  13. 1 2 3 4 5 6 Taylor A, Yardley C (2014). "Advocacy, education, and the role of not-for-profit organizations in Lewy body dementias". Alzheimers Res Ther (Review). 6 (5): 59. doi:10.1186/s13195-014-0059-0. PMC 4468791. PMID 26082807.
  14. ↑ "Lewy body dementia: Hope through research". National Institute of Neurological Disorders and Stroke. US National Institutes of Health. December 8, 2017. Retrieved April 6, 2018.
  15. 1 2 Gallman S (November 4, 2015). "Robin Williams' widow speaks: Depression didn't kill my husband". CNN. Archived from the original on April 11, 2015. Retrieved April 6, 2018.
  16. 1 2 Williams SS (September 2016). "The terrorist inside my husband's brain". Neurology. 87 (13): 1308–11. doi:10.1212/WNL.0000000000003162. PMID 27672165.
  17. 1 2 3 Sahlas DJ (June 2003). "Dementia with Lewy bodies and the neurobehavioral decline of Mervyn Peake". Arch. Neurol. 60 (6): 889–92. doi:10.1001/archneur.60.6.889. PMID 12810496.
  18. ↑ Robbins R (September 30, 2016). "How Lewy body dementia gripped Robin Williams". Scientific American. Retrieved April 9, 2018.
  19. ↑ 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 April 11, 2016. Retrieved April 6, 2018.
  20. 1 2 "LBDA Clarifies Autopsy Report on Comedian, Robin Williams". Lewy Body Dementia Association. November 10, 2014. Retrieved April 19, 2018.
  21. ↑ Carlson M (July 24, 2008). "Obituary: Estelle Getty". theguardian.com. Archived from the original on September 2, 2013. Retrieved October 13, 2013.
  22. ↑ McLellan D (April 23, 2012). "Nicholas King dies at 79; actor helped preserve the Watts Towers". The Los Angeles Times. Retrieved April 19, 2018.
  23. ↑ Dangremond S (May 23, 2017). "Actress and philanthropist Dina Merrill dies at 93". Town and Country Magazine. Retrieved March 22, 2018.
  24. ↑ Woo E (June 24, 2015). "Don Featherstone dies at 79; creator of the plastic pink flamingo". Los Angeles Times. Retrieved March 22, 2018.
  25. ↑ Caffrey J (April 18, 2016). "Casey Kasem and a lesson about end-of-life care". CNN. Retrieved March 22, 2018.
  26. ↑ Papineau P (June 23, 2016). "L'idole d'une génération s'éteint" (in French). Le Devoir. Retrieved April 9, 2018.
  27. ↑ Belanger C (June 22, 2016). "Pierre Lalonde souffrait aussi de la démence à corps de Lewy" (in French). Le Journal de Montréal. Retrieved March 22, 2018.
  28. ↑ "Seymour Berry, 86; Headed U.S. Agency". Washington Post. December 27, 2008. Retrieved April 19, 2018.
  29. ↑ 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. Retrieved July 23, 2008.
  30. ↑ "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. Retrieved April 19, 2018.
  31. ↑ Deerwester J (September 28, 2018). "Ted Turner has Lewy Body Dementia". USA TODAY. Retrieved September 30, 2018.
  32. ↑ O'Donnell M (March 26, 2018). "Arnold R. Hirsch dies; analyzed Chicago segregation in influential book". Chicago Sun Times. Retrieved April 19, 2018.
  33. ↑ "Jessie Isabelle Price Dies On November 12". The Southampton Press. November 23, 2015. Retrieved April 19, 2018.
  34. ↑ Kuc C (June 15, 2015). "For Stan Mikita, all the Blackhawks memories are gone". Chicago Tribune. Archived from the original on June 16, 2015. Retrieved March 22, 2018.
  35. ↑ Wood B (April 6, 2016). "Utah doctor: Diagnosing Parkinson's, Lewy body dementia has improved, but cure remains out of reach". Salt Lake Tribune. Retrieved April 6, 2018.
  36. ↑ Weber B (January 7, 2012). "Andy Carey, Third Baseman for 1950s Yankees, Dies at 80". Retrieved April 19, 2018.
  37. ↑ "Sleepwalk with Me: Comedian's sleep disorder experience comes to film". American Academy of Sleep Medicine. January 26, 2012. Retrieved April 22, 2018.
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