Lewy Body Dementia
What is Lewy body dementia (LBD)?
Lewy body dementia (LBD), is a brain disorder in which proteins, called alpha-synucleins, build up inside certain neurons (brain cells). These clumps of proteins, called Lewy bodies, cause damage to neurons in areas of the brain that affect mental capabilities, behavior, movement and sleep.
In elderly patients, LBD is one of the most common causes of dementia. The symptoms of LBD may closely resemble those of other neurological disorders, including Alzheimer’s disease and Parkinson’s disease.
Doctors do not know why you or your loved one develop LBD while others do not. There is no cure for LBD, but your symptoms can be managed with certain medications, like cholinesterase inhibitors (Aricept®, Exelon®, Razadyne®) and levodopa. You or your loved one may also benefit from non-medical treatments like physical therapy and speech therapy.
Who is most at risk for getting Lewy body dementia (LBD)?
While anyone can develop Lewy body dementia (LBD), those most at risk are over the age of 50. Men are slightly more likely to develop LBD than women.
How common is Lewy body dementia (LBD)?
Lewy body dementia (LBD) is one of the most common causes of dementia in older people. It is estimated that as many as 1.4 million people live with this disorder in the U.S. Most diagnoses are in people over the age of 50.
Symptoms and Causes
What causes Lewy body dementia (LBD)?
Lewy body dementia (LBD) is a broad term covering two separate neurological disorders: dementia with Lewy bodies and Parkinson’s disease dementia. The same biological changes to the brain cause both disorders.
A buildup of Lewy bodies (proteins called alpha-synucleins) causes LBD. Lewy bodies build up in neurons located in certain areas of the brain that are responsible for behavior, movement, and cognitive ability.
Doctors do not know why you or your loved one develop LBD while others do not. There is some thought that the combination of mutation in a person’s genes, environmental risk factors and natural aging might lead to the development of LBD in some people. Research into specific causes is ongoing.
What are the symptoms of Lewy body dementia (LBD)?
Lewy body dementia (LBD) symptoms may resemble those of other neurological disorders, like Alzheimer’s disease and Parkinson’s disease. LBD affects each person differently, and symptoms vary in severity.
Common symptoms of LBD include:
- Decline in thinking, including memory, planning, problem solving, decision making and ability to focus and to understand information in visual form.
- Visual hallucinations, or seeing things that are not there.
- Reduced alertness, attention and ability to concentrate.
- Parkinsonism, a movement disorder with symptoms including slowness, tremors, stiffness, balance problems, soft voice, difficulty swallowing, reduced facial expression and shuffling walk.
- Visuospatial difficulties, including decreased depth perception, trouble recognizing familiar objects and impaired hand-eye coordination.
- Delusions, or beliefs with no basis in reality.
- Changes in behavior and mood including anxiety, agitation, aggression, apathy, depression and paranoia.
- Changes in sleep patterns.
Other symptoms include:
- Acting out while sleeping. Your loved one may act out their dreams during a phase of sleep cycle called rapid eye movement (REM). Sometimes this happens years before their LBD diagnosis. Often called REM sleep behavior disorder (RBD), this condition is described as frequent movements, such as flailing or punching, with yelling or speaking while sleeping. People living with RBD often have difficulty separating dreams from reality when they wake up.
- Changes in normal body functions. Body temperature may waver, blood pressure may fluctuate (causing fainting episodes and falls) and loss of bowel and bladder control.
Diagnosis and Tests
How is Lewy body dementia (LDB) diagnosed?
There are no medical tests that can diagnose Lewy body dementia (LBD) with 100% accuracy. Specialists, including neurologists, geriatric psychiatrists, neuropsychologists and geriatricians, make the diagnosis of ‘probable LBD’ based on the combined results of tests and patient symptoms.
Your healthcare provider will perform a thorough neurological and physical examination. You or your loved one will also complete mental status and neuropsychological tests. These tests check thinking abilities, including memory, word-finding, attention and visual-spatial skills. Your doctor will ask you and your family about your mental status and the history of your symptoms. Be sure to tell your healthcare provider of any physical, cognitive, memory, emotional, behavioral, movement, sleep or physical changes you or your loved one is having. Also, tell your healthcare provider about any of your current medications, supplements, vitamins, herbal products and frequently used over-the-counter products. These will be reviewed to see if they might be a cause of your or your loved one’s symptoms.
Depending on your symptoms, your doctor may also test your blood. If your doctor needs more information, brain imaging studies may be performed.
If you have cognitive deficiency severe enough to impair daily life (dementia) in the presence of any following clinical features, your doctor may suspect diagnosis of LBD:
- Changes in cognition, attention and alertness.
- REM sleep behavior disorder.
- Visual hallucinations.
- Movement problems (Parkinsonism).
Some patients with Parkinson’s disease who develop cognitive symptoms more than a year after the onset of movement problems may be diagnosed with Lewy body dementia.
Can imaging tests diagnose Lewy body dementia (LBD)?
Imaging tests, such as computed tomography (CT) or magnetic resonance imaging (MRI), are done to rule out other causes of dementia such as brain tumors, brain bleeds, stroke, hydrocephalus or other structural causes. Imaging studies for Lewy body dementia (LBD) are usually normal. The only way to make an absolute diagnosis of LBD is by examining the brain at autopsy.
Management and Treatment
How is Lewy body dementia (LBD) treated? Is there a cure?
There is no cure for Lewy body dementia (LBD). Medications and nonmedical therapies, like physical, occupational and speech therapies, manage symptoms as much as possible.
Medications called cholinesterase inhibitors (Aricept®, Exelon®, Razadyne®) help manage the cognitive symptoms of LBD. Memantine (Namenda®) may also be helpful. Symptoms of Parkinsonism, like tremors, are usually treated with levodopa, a drug commonly used to treat Parkinson’s disease.
You or your loved one may also benefit from treatments like physical therapy or speech therapy. These treatments help retain physical function and improve muscle strength.
What complications are associated with medications used to treat Lewy body dementia (LBD)?
Up to 50% of people living with Lewy body dementia (LBD) can have severe side effects when treated with certain antipsychotic medications. These are known as the typical or traditional antipsychotics and include such drugs as thoridazine, haloperidol, chlorpromazine and perphenazine. This class of older, first-generation antipsychotics can cause sedation and make cognitive symptoms and movement problems (Parkinsonism) worse. A life-threatening reaction to an antipsychotic medication, called neuroleptic malignant syndrome, is possible. Symptoms include rigid muscles, changing blood pressure, high fever, confusion and fast heart rate. Contact your healthcare provider immediately if you or your loved are taking an antipsychotic and develop these symptoms.
Visual hallucinations and behavioral changes may be treated with the newer, atypical antipsychotic medications pimavanserin (Nuplazid®), quetiapine (Seroquel®) or clozapine (Clozaril®). However, because all antipsychotic medications – both older, typical medications and newer atypical medications – can increase the risk of death in elderly patients with dementia, you and your healthcare provider should carefully discuss the risks and benefits and using these medications.
Other medications, like antidepressants or sedative antihistamines, may increase confusion in people with LBD.
Talk with your healthcare provider about all the medications you or your loved one takes. Many drugs have side effects that can worsen symptoms or add to them. If medications to treat symptoms are not working to decrease symptoms, others can be tried. Never hesitate to talk to your healthcare with your concerns or to ask questions.
Can Lewy body dementia (LBD) be prevented?
Currently, there is no known way to prevent Lewy body dementia (LBD).
Outlook / Prognosis
How do lewy bodies actually cause harm in the brain?
Lewy bodies are clumps of a specific protein (alpha synuclein) that build up in the brain. The lewy bodies form in areas in the brain involved in memory, thinking and movement. Technically, the lewy bodies overwhelm brain cells’ (neurons) normal biologic functions, causing cells to die.
What can I expect if I or my loved one have a diagnosis of Lewy body dementia (LBD)?
Each person’s experience with Lewy body dementia (LBD) is unique to them. How slowly or quickly the disease progresses is impossible to know, but may be influenced by your general health and any existing diseases you may have. Because LBD is a progressive disease, difficulties with mind and body functions get worse over time. Currently, there is no known way to stop the progression of the disease. After diagnosis, most people with LBD live between five and seven years. Some people with LBD live up to 20 years after their diagnosis.
However, there’s always hope. Research on LBD, dementia with lewy bodies, Alzheimer’s disease, Parkinson’s disease with dementia are ongoing. New medications are being developed and new approaches to treatment are being investigated.
How can a person diagnosed with Lewy body dementia live the best life possible?
First, it’s important to interact with others as much as possible. Plus exercise and eat a healthy diet to keep mind and body as strong as possible.
Use music and aromatherapy to reduce anxiety and improve mood.
Other things you can do involve seeking the help and assistance of others. Therapists can improve the quality of life of someone living with Lewy body dementia. Therapists include:
- Physical therapists: These therapists can help improve physical strength, flexibility, balance and walking mechanics.
- Occupational therapists: These therapists can teach skills and methods to maintain your ability to perform activities of everyday life and remain independent.
- Speech therapists: These therapists can improve swallowing difficulties and ability to speak more clearly.
Persons with Lewy body dementia and their families can also benefit from counseling with a psychotherapist. This counselor teaches how to manage emotional and behavioral problems. Finally, joining a support group – there are support groups for persons with LBD and for the caregivers of persons with LBD. Support groups help solve day-to-day problems and frustrations through sharing similar experiences. See the resource section of this article for links to support groups.
Is Lewy body dementia an inherited condition?
One of the more recent discoveries toward identifying a cause of Lewy body dementia is the finding of an increasing number of gene mutations. Two genetic risk factors recently discovered are variants in the APOE and GBA genes. APOE is already known to increase the risk of developing Alzheimer’s disease. There is growing evidence that it also increases the risk for dementia with lewy bodies. Similarly, the GBA gene increases the risk for both Parkinson’s disease and dementia with lewy bodies. Despite these findings, genetic changes as a cause of LBD are still considered rare by scientists. Most cases of Lewy body dementia are not thought to be inherited.
Genetic testing for routine screening for LBD is not currently recommended. Discuss the pros and cons of testing with your healthcare providers if you have a family history of multiple members with Parkinson’s disease and/or dementia with lewy bodies.
How exactly is Lewy body dementia related to Alzheimer’s disease and Parkinson’s disease?
Lewy body dementia is a broad, general term for dementia in which lewy bodies are present in the brain. Dementia with lewy bodies and Parkinson’s disease dementia are two related clinical disorders that make up the general broader category of Lewy body dementia. Sometimes LBD is first diagnosed as Parkinson’s disease or Alzheimer’s disease based on its symptoms.
- Parkinson’s disease dementia (PDD): You might be diagnosed with Parkinson’s disease if you start out with a movement disorder typical to Parkinson’s but then have your diagnosis changed to PDD when dementia symptoms develop.
- Alzheimer’s disease (AD): You might start out with memory or cognitive disorder that leads to a diagnosis of AD. Over time, other distinctive symptoms begin to appear and your diagnosis is then changed to dementia with lewy bodies. Distinctive symptoms of LBD include the changes in attention, alertness and cognitive ability; changes in walking and movement; visual hallucinations; REM sleep behavior disorder and severe sensitivity to some antipsychotics used to treat hallucinations.
When should I call my doctor?
If you develop symptoms of Lewy body disease, contact your doctor for a thorough evaluation. Your doctor will develop a treatment plan based on your diagnosis and symptoms.
What resources are available to help people with Lewy body dementia (LBD)?
If you or one of your family members or friends have Lewy body dementia (LBD), you might benefit from seeking additional help. Here are some places to contact:
- Lewy Body Dementia Association. Find Support.
- Administration for Community Living in partner with the Administration on Aging. Eldercare Locator.
- Family Caregiver Alliance, National Center on Caregiving
- Well Spouse Association
- National Institute on Aging. (search for Lewy body dementia)
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