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]

Not to be confused with dementia with Lewy bodies, one of the Lewy body dementias, along with Parkinson's disease dementia.

Lewy bodies

Lewy bodies, abnormal aggregations of protein that develop inside nerve cells, were first described by Friedrich Heinrich Lewy. Lewy identified the structures in the brains of deceased Parkinson's patients in 1910-1911, and published his findings in 1912.[5] They were named after Lewy in 1938 by R. Hassler.[6][7]

Signs and symptoms
Further information: Dementia with Lewy bodies § 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 behaviour disorder (RBD), in which individuals lose normal muscle paralysis during REM sleep, and act out their dreams.[8][9] Other frequent symptoms include visual hallucinations; marked fluctuations in attention or alertness; and slowness of movement, trouble walking, or rigidity.[8] The autonomic nervous system is usually affected, and can result in symptoms such as changes in blood pressure, heart and gastrointestinal function, urinary incontinence, and constipation.[10] Mood changes such as depression, apathy, and anxiety are common.[8]

RBD may appear decades before any other symptoms.[9] On autopsy, 94 to 98% of individuals with polysomnography-confirmed RBD are found to have a synucleinopathy—most commonly DLB or Parkinson's disease,[11][12][13] in about equal proportions.[14] 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.[11]

Diagnosis
Further information: Dementia with Lewy bodies § Diagnosis

While both dementia with Lewy bodies and Parkinson's disease dementia have similar neuropathologic features, they are highly variable and should not be diagnosed on pathologic features alone.[15] Generally, DLB is distinguished from PDD 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.[8] Symptoms of Lewy body dementia can sometimes be confused with other neurodegenerative diseases, such as Alzheimer's, or psychiatric disorders like schizophrenia. [16]

Pathophysiology
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.[17] Lewy bodies and Lewy neurites have been found to develop from aggregation of misfolded alpha-synuclein, a protein thought to assist in neurotransmitter release and vesicle turnover. However, no definitive link between Lewy bodies and neurodegenerative effects has been found.[15] Despite differences in the timing of the appearance of symptoms, the two dementias "show remarkably convergent neuropathological changes at autopsy".[2]

Management

Current disease management focuses on treating each symptom.[18] For cognitive symptoms, the standard treatment is Cholinesterase Inhibitors (CHEIs). This drug class is effective in reducing apathy, visual hallucinations, and delusions symptoms.[19] For movement symptoms, Levodopa is considered the standard treatment.[18] Some research efforts in exploring alternative LBD therapeutics have been called questionable due to a variety of factors including choice of study design, exclusion criteria, limitation of outcomes, strength of evidence, and complications between PDD and DLB classifications.[20] Beside pharmacological management, there are other available non-medical treatments like physical therapy, speech therapy, occupational therapy, individual and family psychotherapy, and support groups that might be recommended by physicians to improve and address the needs of each patient.[18]

Epidemiology

As of 2013, Lewy body dementias affect about 1.3 million people in the United States, though this number is said to be an underestimate given the difficulty of diagnosing the disorder.[21] LBD usually develops after the age of 50.[22] While a study published on JAMA Neurology in 2013 pointed out a notable difference between DLB and PDD in age and sex, more research is needed to confirm any discrepancies.[21]

Society and culture

Advocacy and awareness

Lewy body dementias are more often misdiagnosed than any other common dementia.[23] 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.[23] 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.[23] 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.[23] 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.[23]

Notable individuals
His widow said Robin Williams (shown in 2011) was diagnosed on autopsy with Lewy bodies.[24][25]

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.[26] Sahlas said his death was "variously ascribed to Alzheimer disease, Parkinson disease, or postencephalitic parkinsonism".[26] 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.[26]

Robin Williams, the American actor and comedian, committed suicide on August 11, 2014. He had been diagnosed with Parkinson's disease,[24] and according to his widow, he experienced depression, anxiety, and increasing paranoia.[25] Upon autopsy, his widow said he was found to have diffuse DLB.[27] 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.[28] 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.[29] According to Dennis Dickson, the LBDA spokesman, "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."[29]

Other entertainers and artists who have or died from LBD include Estelle Getty, an actress known for her role in the television series The Golden Girls,[30] Nicholas King, a US actor and horticulturist,[31] actress Dina Merrill,[32] Donald Featherstone, who created the plastic pink flamingo,[33] American radio and television host Casey Kasem,[34] and Canadian singer Pierre Lalonde.[35][36]

Individuals from industry or government who had or died from LBD are Seymour Berry, US Director of the Bureau of Engraving and Printing,[37] Los Angeles Times publisher Otis Chandler,[38] Philip J. Rock, a US Democratic politician of the Illinois Senate,[39] and U.S. media mogul and philanthropist Ted Turner.[40]

Arnold R. Hirsch, a US historian who taught at the University of New Orleans,[41] and Jessie Isabelle Price, a US veterinary microbiologist,[42] died from LBD.

In the sports realm, Stan Mikita, Canadian ice hockey player,[43] and Jerry Sloan, American professional basketball player and coach,[44] have been diagnosed with DLB. Andy Carey, a US Major League Baseball player for the New York Yankees,[45] and Bill Buckner, also an MLB player, died of LBD.[46]

In film

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.[47]

References
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