Description Dementia with Lewy bodies is a nervous system disorder characterized by a decline in intellectual function dementia , a group of movement problems known as parkinsonism, visual hallucinations, sudden changes fluctuations in behavior and intellectual ability, and acting out dreams while asleep REM sleep behavior disorder.
Frequency Dementia with Lewy bodies is estimated to affect 1. Inheritance When dementia with Lewy bodies is caused by SNCA or SNCB gene mutations, it is inherited in an autosomal dominant pattern , which means one copy of the altered gene in each cell is sufficient to cause the disorder.
Research Studies from ClinicalTrials. Epub Aug Inclusion of RBD improves the diagnostic classification of dementia with Lewy bodies. Can J Neurol Sci. Epub Jun 7.
Clinical presentation and differential diagnosis of dementia with Lewy bodies: a review. Int J Geriatr Psychiatry. Epub Oct A multicenter study of glucocerebrosidase mutations in dementia with Lewy bodies. JAMA Neurol. Gene expression profiling of brain samples from patients with Lewy body dementia. Biochem Biophys Res Commun. Epub Sep GBA mutations increase risk for Lewy body disease with and without Alzheimer disease pathology.
Despite the benefits offered by available treatments, there is deterioration in cognitive and motor function over time.
We are currently involved in a study evaluating a caregiver support model for all types of dementia, including Lewy body dementia. We are also in the early stages of bringing a clinical trial for Lewy body dementia to UVA. It will test a new treatment, and UVA will be one site in a multi-site trial.
November 19, Jane Kelly , jak4g virginia. Several drugs and other treatments are available to treat LBD symptoms.
It is important to work with a knowledgeable health professional because certain medications can make some symptoms worse. Some medications used to treat Alzheimer's disease also may be used to treat the cognitive symptoms of LBD. These drugs, called cholinesterase inhibitors, act on a chemical in the brain that is important for memory and thinking.
They may also improve hallucinations, apathy, and delusions. The U. Food and Drug Administration has approved one Alzheimer's drug, rivastigmine, to treat cognitive symptoms in Parkinson's disease dementia. Several other drugs are being tested as possible treatments for LBD symptoms or to disrupt the underlying disease process. LBD-related movement symptoms may be treated with medications used for Parkinson's disease , called carbidopa-levodopa.
These drugs can help make it easier to walk, get out of bed, and move around. However, they cannot stop or reverse the disease itself. Side effects of this medication can include hallucinations and other psychiatric or behavioral problems. Because of this risk, physicians may recommend not treating mild movement symptoms with medication.
Other Parkinson's medications are less commonly used in people with LBD due to a higher frequency of side effects. People with LBD may benefit from physical therapy and exercise. Talk with your doctor about what physical activities are best. Sleep problems may increase confusion and behavioral problems in people with LBD and add to a caregiver's burden.
A physician can order a sleep study to identify any underlying sleep disorders such as sleep apnea , restless leg syndrome, and REM sleep behavior disorder. REM sleep behavior disorder, a common LBD symptom, involves acting out one's dreams, leading to lost sleep and even injuries to individuals and their sleep partners.
Clonazepam, a drug used to control seizures and relieve panic attacks, is often effective for the disorder at very low dosages. However, it can have side effects such as dizziness, unsteadiness, and problems with thinking. Melatonin, a naturally occurring hormone used to treat insomnia, may also offer some benefit when taken alone or with clonazepam. Excessive daytime sleepiness is also common in LBD. If it is severe, a sleep specialist may prescribe a stimulant to help the person stay awake during the day.
Some people with LBD have difficulty falling asleep. If trouble sleeping at night persists, a physician may recommend a prescription medication. It is important to note that treating insomnia and other sleep problems in people with LBD has not been extensively studied, and that treatments may worsen daytime sleepiness and should be used with caution.
Sleep problems can also be addressed by avoiding lengthy naps, increasing daytime exercise, and avoiding caffeine, alcohol, and chocolate late in the day.
Behavioral and mood problems in people with LBD can arise from hallucinations, delusions, pain , illness, stress, or anxiety. They may also be the result of frustration, fear, or feeling overwhelmed. The person may resist care or lash out verbally or physically.
Medications are appropriate if the behavior interferes with the person's care or the safety of the person or others. If medication is used, then the lowest possible dose for the shortest period of time is recommended.
The first step is to visit a doctor to see if a medical condition unrelated to LBD is causing the problem. Injuries, fever, urinary tract or pulmonary infections, pressure ulcers bed sores , and constipation can worsen behavioral problems and increase confusion. Certain medications, such as anticholinergics and antihistamines may also cause behavioral problems.
For example, some medications for sleep problems, pain, bladder control, and LBD-related movement symptoms can cause confusion, agitation, hallucinations, and delusions. Similarly, some anti-anxiety medicines can actually increase anxiety in people with LBD.
Several participants described that the individual stopped breathing for a minute and the family thought that was the end, but then the individual started breathing again. Participants described preparing enjoyable outings prior to the final days of life, such as using a wheelchair to spend time outdoors. Numerous participants described value from visiting hospice musicians.
Family members played favorite music recordings or television shows. Friends and volunteers read books aloud or recited poetry. Massages and pools helped some individuals. One family printed large photos and placed them around the room. Several participants reported difficulties accessing hospice or not understanding its benefits. Hospice experiences were usually but not universally positive. Morphine and benzodiazepines commonly helped, but some individuals had negative reactions.
Several individuals received antipsychotics, with a severe reaction occurring in one case. Individuals with DLB were often ready for death by the end.
Death was peaceful for most participants. The emphasis on lack of knowledge is consistent with existing literature. Knowing what to expect is an unmet need described by caregivers in various dementias [ 13 — 16 ]. Many families are unaware that dementia can be terminal [ 15 , 17 , 20 ]. Recognizing EOL in individuals with dementia requires technical skills, systematic approaches, observation, and knowledge for interpreting signs of deterioration [ 21 ]. This can be challenging for physicians without DLB experience and most individuals with DLB receive dementia care from primary care physicians [ 22 ].
Advanced dementia features include profound cognitive deficits, minimal verbal abilities, loss of independent ambulation, inability to perform activities of daily living ADLs , and urinary and fecal incontinence [ 23 ]. The ADEPT score for predicting 6-month mortality in dementia uses risk factors including age, gender, shortness of breath, pressure ulcers, inability to perform ADLs, bed-bound status, insufficient oral intake, recent weight loss, body mass index, and congestive heart failure to guide prediction [ 25 ].
Interview participants described many of these features in the months approaching EOL. Individuals with DLB may also have DLB-specific symptoms such as worsened hallucinations, cognitive fluctuations, parkinsonism Table 1 and antipsychotic hypersensitivity. Antipsychotic hypersensitivity is a unique DLB feature [ 26 , 27 ] that is critical to incorporate into hospice planning to avoid reactions that may worsen EOL experiences.
ACP is part of dementia quality measures [ 12 ]. Proactive ACP allows active patient involvement and is an important part of dementia care [ 28 ]. ACP also improved the quality of dying of nursing home residents with dementia [ 29 ]. This is consistent with current results, where ACP improved patient and caregiver quality of life and families without ACP expressed regret.
Two participants raised issues surrounding euthanasia in dementia. This is a complex ethical issue [ 30 ] outside the scope of the current study, but emphasizes the need for palliative care targeting quality of life and dignity and hospice providing comfort, support, and symptom control. Despite a clear role for palliative care throughout DLB and hospice care at the EOL, many participants voiced lack of knowledge regarding hospice and lack of education and assistance from their physicians.
This may reflect a more general lack of public and caregiver knowledge regarding palliative care [ 31 ]. Lack of family and physician knowledge are known barriers to hospice care in dementia [ 20 ] and caregivers report difficulty accessing hospice for family members with dementia [ 32 ]. Hospice is commonly initiated only in the last weeks of life in DLB [ 5 ] and other dementias [ 32 , 33 ]. Most dementia caregivers describe positive hospice interactions, but some were disappointed by lacking guidance, responsiveness, or individual interactions [ 32 ], similar to current findings.
Death is often viewed as a relief by patients and caregivers [ 32 ]. Most individuals with dementia receiving EOL care in long-term care facilities hospice die peacefully [ 35 ], but current participants reported distress relating to the duration and unpredictability of the dying process and lack of communication that this was to be expected. Prior research also shows poor communication between physicians and caregivers at the EOL [ 36 , 37 ]. Such education needs to start with outpatient physicians and continue with skilled nursing home and hospice teams and cover a variety of EOL topics Table 3.
Given that many physicians caring for individuals with DLB do not have experience on which to base counseling, identifying common experiences is critical to inform the education needed by patients and caregivers. All participants were U. For example, accessibility of palliative care and hospice will vary by country.
Despite sampling to increase male representation, most participants were women, consistent with dementia caregiver statistics in the U. Demographics other than gender and role were not collected. Participants may have volunteered because of particular experiences or strong views, but identified themes were consistent with published dementia research. Recall bias was limited by recruiting only individuals whose loved one died in the prior 5 years.
COREQ item checklist outlining the page where each element of qualitative research is reported. Staff from the LBDA participated in data collection, analysis, and preparation of the manuscript. MJA previously received research support from the Michael J. Fox Foundation. MJA receives compensation from the American Academy of Neurology for work as an evidence-based medicine methodology consultant and has participated as faculty in the annual meeting of the American Academy of Neurology and for Medscape CME.
These funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. National Center for Biotechnology Information , U.
PLoS One. Published online May Melissa J. James E. Alessandra Solari, Editor. Author information Article notes Copyright and License information Disclaimer. Competing Interests: S. Alliance has declared that no competing exists. Taylor and P. She receives compensation from the American Academy of Neurology for work as an evidence-based medicine methodology consultant and has participated as faculty in the annual meeting of the American Academy of Neurology and for Medscape CME.
Received Nov 20; Accepted May 5. This is an open access article distributed under the terms of the Creative Commons Attribution License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. This article has been cited by other articles in PMC. S1 File: Semi-structured interview guide. S1 Appendix: Qualitative coding. Coding tables with additional exemplar quotes. Abstract Background Dementia caregivers describe knowing what to expect as an unmet need and many are unaware that dementia can be a terminal condition.
Method We conducted telephone interviews with caregivers and family members of individuals who died with DLB in the last 5 years using a semi-structured questionnaire to identify and describe EOL experiences. Results Thirty individuals participated in interviews. End-of-life time course Families described new or worsened symptoms in the last weeks and months of life Table 1. Table 1 Reported symptoms in the end-of-life period.
And I ended up feeding him all three of his meals every day, and encouraged him and little by little still he wouldn't eat them. You know, he- he—the rest of the time it was just unintelligible noises. I mean it was just so rapid. He was seeing people. Open in a separate window.
Lack of family understanding Multiple participants described challenges arising from family member lack of understanding. Hospice Participants described not understanding what hospice is or how it could help. One participant was frustrated by the loss of services associated with hospice: I really fought not to put him on hospice because I felt like he should have as much therapy and PT [physical therapy] and—- good experiences as possible and not just let him have end of life experience before he was ready.
Views regarding right-to-die Multiple participants stressed the importance of dying with dignity and respect. Medications at the end of life Many participants described stopping DLB medications e.
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