What is Lewy Body dementia ?

What Is Lewy Body Dementia ?

Lewy body dementia is a disease associated with abnormal deposits of a protein called alpha-synuclein in the brain ¹. These deposits, called Lewy bodies, affect chemicals in the brain whose changes, in turn, can lead to problems with thinking, movement, behavior, and mood. Lewy body dementia is one of the most common causes of dementia, after Alzheimer’s disease and vascular disease dementia. People affected by this condition may experience a variety of symptoms such as changes in alertness and attention; hallucinations; problems with movement and posture; muscle stiffness; confusion; and/or memory loss ².

Lewy body dementias are the second most common form of degenerative dementia. The only other form of degenerative dementia that is more common than Lewy body dementia is Alzheimer’s disease ³. Lewy body dementias affect an estimated 1.4 million individuals and their families in the United States. And currently there is no cure for Lewy body dementia ⁴.

Lewy body dementia is an umbrella term for dementia associated with the presence of Lewy bodies (abnormal deposits of a protein called alpha-synuclein) in the brain.

Lewy body dementia usually occurs sporadically in people with no family history of the condition. Rarely, more than one family member may be affected. There is currently no cure for Lewy body dementia; however, medications may be available to help manage the associated symptoms ⁵, ⁶, ⁷.

Diagnosing Lewy body dementia can be challenging for a number of reasons. Early Lewy body dementia symptoms are often confused with similar symptoms found in other brain diseases like Alzheimer’s disease. Also, Lewy body dementia can occur alone or along with Alzheimer’s or Parkinson’s disease.

There are two types of Lewy body dementia:

1. Dementia with Lewy bodies and
2. Parkinson’s disease dementia.

The earliest signs of these two diseases differ but reflect the same biological changes in the brain. Over time, people with dementia with Lewy bodies or Parkinson’s disease dementia may develop similar symptoms.

Lewy body dementia can have three common presentations ³:

Regardless of the initial symptom, over time all three presentations of Lewy body dementia will develop very similar cognitive, physical, sleep and behavioral features.

1. Some individuals will start out with a movement disorder leading to the diagnosis of Parkinson’s disease and later develop dementia. This is diagnosed as Parkinson’s disease dementia.
2. Another group of individuals will start out with a cognitive/memory disorder that may be mistaken for Alzheimer’s disease, but over time two or more distinctive features become apparent leading to the diagnosis of ‘dementia with Lewy bodies’ (DLB).
3. Lastly, a small group will first present with neuropsychiatric symptoms, which can include hallucinations, behavioral problems, and difficulty with complex mental activities, also leading to an initial diagnosis of dementia with Lewy bodies.

What Are Lewy Bodies ?

Lewy bodies are named for Dr. Friederich Lewy, a German neurologist. In 1912, he discovered abnormal protein deposits that disrupt the brain’s normal functioning in people with Parkinson’s disease ¹. These abnormal deposits are now called “Lewy bodies.”

Lewy bodies are made of a protein called alpha-synuclein ¹. In the healthy brain, alpha-synuclein plays a number of important roles in neurons (nerve cells) in the brain, especially at synapses, where brain cells communicate with each other. In Lewy body dementia, alpha-synuclein forms into clumps inside neurons, starting in particular regions of the brain. This process causes neurons to work less effectively and, eventually, to die. The activities of brain chemicals important to brain function are also affected. The result is widespread damage to certain parts of the brain and a decline in abilities affected by those brain regions.

Lewy bodies affect several different brain regions in Lewy body dementia:

• The cerebral cortex, which controls many functions, including information processing, perception, thought, and language
• The limbic cortex, which plays a major role in emotions and behavior
• The hippocampus, which is essential to forming new memories
• The midbrain, including the substantia nigra, which is involved in movement
• The brain stem, which is important in regulating sleep and maintaining alertness
• Brain regions important in recognizing smells (olfactory pathways)

Who Is Affected by Lewy body dementia ?

Lewy body dementia affects more than 1 million individuals in the United States ¹. Lewy body dementia typically begins at age 50 or older, although sometimes younger people have it. Lewy body dementia appears to affect slightly more men than women.

Lewy body dementia is a progressive disease, meaning symptoms start slowly and worsen over time. The disease lasts an average of 5 to 7 years from the time of diagnosis to death, but the time span can range from 2 to 20 years ¹. How quickly symptoms develop and change varies greatly from person to person, depending on overall health, age, and severity of symptoms.

In the early stages of Lewy body dementia, usually before a diagnosis is made, symptoms can be mild, and people can function fairly normally. As the disease advances, people with Lewy body dementia require more and more help due to a decline in thinking and movement abilities. In the later stages of the disease, they may depend entirely on others for assistance and care.

Some Lewy body dementia symptoms may respond to treatment for a period of time. Currently, there is no cure for the disease ¹. Research is improving our understanding of this challenging condition, and advances in science may one day lead to better diagnosis, improved care, and new treatments.

What Causes Lewy Body Dementia ?

The precise cause of Lewy body dementia is unknown, but scientists are learning more about its biology and genetics ⁸. For example, they know that an accumulation of Lewy bodies is associated with a loss of certain neurons in the brain that produce two important neurotransmitters, chemicals that act as messengers between brain cells. One of these messengers, acetylcholine, is important for memory and learning. The other, dopamine, plays an important role in behavior, cognition, movement, motivation, sleep, and mood. Other brain changes, including the amyloid plaques and tau tangles found in people with Alzheimer’s disease, may also occur.

Age is considered the greatest risk factor. Most people who develop the disorder are over age 50 ⁸.

Other known risk factors for Lewy body dementia include the following:

• Diseases and health conditions—Certain diseases and health conditions, particularly Parkinson’s disease and REM sleep behavior disorder, are linked to a higher risk of Lewy body dementia.

• Genetics—While having a family member with Lewy body dementia may increase a person’s risk, Lewy body dementia is not normally considered a genetic disease. A small percentage of families with dementia with Lewy bodies has a genetic association, such as a variant of the GBA gene, but in most cases, the cause is unknown. At this time, no genetic test can accurately predict whether someone will develop Lewy body dementia. Future genetic research may reveal more information about causes and risk.

• Lifestyle—No specific lifestyle factor has been proven to increase one’s risk for Lewy body dementia. However, some studies suggest that a healthy lifestyle—including regular exercise, mental stimulation, and a healthy diet—might reduce the chance of developing age-associated dementias.

How is Lewy body dementia diagnosed ?

It’s important to know which type of Lewy body dementia a person has, both to tailor treatment to particular symptoms and to understand how the disease will likely progress. Clinicians and researchers use the “1-year rule” to diagnose which form of Lewy body dementia a person has. If cognitive symptoms appear within a year of movement problems, the diagnosis is dementia with Lewy bodies. If cognitive problems develop more than a year after the onset of movement problems, the diagnosis is Parkinson’s disease dementia ⁹.

Early recognition, diagnosis and treatment of Lewy body dementia can improve the patients’ quality of life. Lewy body dementia may affect an individual’s cognitive abilities, motor functions, and/or ability to complete activities of daily living.

Regardless of the initial symptoms, over time people with Lewy body dementia often develop similar symptoms due to the presence of Lewy bodies in the brain. But there are some differences. For example, dementia with Lewy bodies may progress more quickly than Parkinson’s disease dementia ⁹.

Dementia with Lewy bodies is often hard to diagnose because its early symptoms may resemble those of Alzheimer’s, Parkinson’s disease, or a psychiatric illness. As a result, it is often misdiagnosed or missed altogether. As additional symptoms appear, it is often easier to make an accurate diagnosis.

The good news is that doctors are increasingly able to diagnose Lewy body dementia earlier and more accurately as researchers identify which symptoms help distinguish it from similar disorders.

Difficult as it is, getting an accurate diagnosis of Lewy body dementia early on is important so that a person:

• Gets the right medical care and avoids potentially harmful treatment
• Has time to plan medical care and arrange legal and financial affairs
• Can build a support team to stay independent and maximize quality of life.

Early and accurate diagnosis is important because Lewy body dementia patients may react to certain medications differently than Alzheimer’s disease or Parkinson’s disease patients. A variety of drugs, including anticholinergics and some antiparkinsonian medications, can worsen Lewy body dementia symptoms ³. Treatment should always be monitored by a physician and may include: prescriptive and other therapies, exercise, diet, sleep habits, changes in behavior and daily routines.

Many traditional antipsychotic medications (for example, haloperidol, thioridazine) are sometimes prescribed for individuals with Alzheimer’s disease and other forms of dementia to control behavioral symptoms. However, Lewy body dementia affects an individual’s brain differently than other dementias. As a result, these medications can cause a severe worsening of movement and a potentially fatal condition known as neuroleptic malignant syndrome (NMS). NMS causes severe fever, muscle rigidity and breakdown that can lead to kidney failure ³.

While a diagnosis of Lewy body dementia can be distressing, some people are relieved to know the reason for their troubling symptoms. It is important to allow time to adjust to the news. Talking about a diagnosis can help shift the focus toward developing a care plan.

Researchers are studying ways to diagnose Lewy body dementia more accurately in the living brain. Certain types of neuroimaging—positron emission tomography and single-photon emission computed tomography—have shown promise in detecting differences between dementia with Lewy bodies and Alzheimer’s disease. These methods may help diagnose certain features of the disorder, such as dopamine deficiencies. Researchers are also investigating the use of lumbar puncture (spinal tap) to measure proteins in cerebrospinal fluid that might distinguish dementia with Lewy bodies from Alzheimer’s disease and other brain disorders.

Talking to both patients and caregivers helps doctors make a diagnosis.

It is important for the patient and a close family member or friend to tell the doctor about any symptoms involving thinking, movement, sleep, behavior, or mood. Also, discuss other health problems and provide a list of all current medications, including prescriptions, over-the-counter drugs, vitamins, and supplements. Certain medications can worsen Lewy body dementia symptoms.

Caregivers may be reluctant to talk about a person’s symptoms when that person is present. Ask to speak with the doctor privately if necessary. The more information a doctor has, the more accurate a diagnosis can be.

What are Symptoms of Lewy Body Dementia ?

People with Lewy body dementia may not have every Lewy body dementia symptom, and the severity of symptoms can vary greatly from person to person ¹⁰. Throughout the course of the disease, any sudden or major change in functional ability or behavior should be reported to a doctor.

The most common symptoms of Lewy body dementia include:

• Impaired thinking, such as loss of executive function (planning, processing information), memory, or the ability to understand visual information.
• Fluctuations in cognition, attention or alertness;
• Problems with movement including tremors, stiffness, slowness and difficulty walking
• Visual hallucinations (seeing things that are not present)
• Sleep disorders, such as acting out one’s dreams while asleep
• Behavioral and mood symptoms, including depression, apathy, anxiety, agitation, delusions or paranoia
• Changes in autonomic body functions, such as blood pressure control, temperature regulation, and bladder and bowel function.

The symptoms of Lewy body dementia are treatable:

All medications prescribed for Lewy body dementia are approved for a course of treatment for symptoms related to other diseases such as Alzheimer’s disease and Parkinson’s disease with dementia and offer symptomatic benefits for cognitive, movement and behavioral problems.

The most common symptoms include changes in cognition, movement, sleep, and behavior ¹⁰.

Cognitive Symptoms

Lewy body dementia causes changes in thinking abilities. These changes may include:

• Dementia—Severe loss of thinking abilities that interferes with a person’s capacity to perform daily activities. Dementia is a primary symptom in Lewy body dementia and usually includes trouble with visual and spatial abilities (judging distance and depth or misidentifying objects), planning, multitasking, problem solving, and reasoning. Memory problems may not be evident at first but often arise as Lewy body dementia progresses. Dementia can also include changes in mood and behavior, poor judgment, loss of initiative, confusion about time and place, and difficulty with language and numbers.

• Cognitive fluctuations—Unpredictable changes in concentration, attention, alertness, and wakefulness from day to day and sometimes throughout the day. A person with Lewy body dementia may stare into space for periods of time, seem drowsy and lethargic, or sleep for several hours during the day despite getting enough sleep the night before. His or her flow of ideas may be disorganized, unclear, or illogical at times. The person may seem better one day, then worse the next day. These cognitive fluctuations are common in Lewy body dementia but are not always easy for a doctor to identify.

• Hallucinations—Seeing or hearing things that are not present. Visual hallucinations occur in up to 80 percent of people with Lewy body dementia, often early on. They are typically realistic and detailed, such as images of children or animals. Auditory hallucinations are less common than visual ones but may also occur. Hallucinations that are not disruptive may not require treatment. However, if they are frightening or dangerous (for example, if the person attempts to fight a perceived intruder), then a doctor may prescribe medication.

Table 1. Main Symptoms of Lewy Body Dementia

Movement Symptoms

Some people with Lewy body dementia may not experience significant movement problems for several years. Others may have them early on. At first, signs of movement problems, such as a change in handwriting, may be very mild and thus overlooked. Parkinsonism is seen early on in Parkinson’s disease dementia but can also develop later on in dementia with Lewy bodies. Specific signs of parkinsonism may include:

• Muscle rigidity or stiffness
• Shuffling gait, slow movement, or frozen stance
• Tremor or shaking, most commonly at rest
• Balance problems and falls
• Stooped posture
• Loss of coordination
• Smaller handwriting than was usual for the person
• Reduced facial expression
• Difficulty swallowing
• A weak voice

Sleep Disorders

Sleep disorders are common in people with Lewy body dementia but are often undiagnosed. A sleep specialist can play an important role on a treatment team, helping to diagnose and treat sleep disorders. Sleep-related disorders seen in people with Lewy body dementia may include:

• REM sleep behavior disorder—A condition in which a person seems to act out dreams. It may include vivid dreaming, talking in one’s sleep, violent movements, or falling out of bed. Sometimes only the bed partner of the person with Lewy body dementia is aware of these symptoms. REM sleep behavior disorder appears in some people years before other Lewy body dementia symptoms.
• Excessive daytime sleepiness—Sleeping 2 or more hours during the day.
• Insomnia—Difficulty falling or staying asleep, or waking up too early.
• Restless leg syndrome—A condition in which a person, while resting, feels the urge to move his or her legs to stop unpleasant or unusual sensations.
• Walking or moving usually relieves the discomfort.

Behavioral and Mood Symptoms

Changes in behavior and mood are possible in Lewy body dementia. These changes may include:

• Depression—A persistent feeling of sadness, inability to enjoy activities, or trouble with sleeping, eating, and other normal activities.
• Apathy—A lack of interest in normal daily activities or events; less social interaction.
• Anxiety—Intense apprehension, uncertainty, or fear about a future event or situation. A person may ask the same questions over and over or be angry or fearful when a loved one is not present.
• Agitation—Restlessness, as seen by pacing, hand wringing, an inability to get settled, constant repeating of words or phrases, or irritability.
• Delusions—Strongly held false beliefs or opinions not based on evidence. For example, a person may think his or her spouse is having an affair or that relatives long dead are still living. Another delusion that may be seen in people with Lewy body dementia is Capgras syndrome, in which the person believes a relative or friend has been replaced by an imposter.
• Paranoia—An extreme, irrational distrust of others, such as suspicion that people are taking or hiding things.

Other Lewy body dementia Symptoms

People with Lewy body dementia can also experience significant changes in the part of the nervous system that regulates automatic functions such as those of the heart, glands, and muscles. The person may have:

• Changes in body temperature
• Problems with blood pressure
• Dizziness
• Fainting
• Frequent falls
• Sensitivity to heat and cold
• Sexual dysfunction
• Urinary incontinence
• Constipation
• A poor sense of smell.

Treatment for Lewy body dementia

There is no cure for Lewy body dementia. Treatments are aimed at controlling the cognitive, psychiatric, and motor symptoms of the disorder. Acetylcholinesterase inhibitors, such as donepezil and rivastigmine, are primarily used to treat the cognitive symptoms of Lewy body dementia, but they may also be of some benefit in reducing the psychiatric and motor symptoms. Doctors tend to avoid prescribing antipsychotics for hallucinatory symptoms of Lewy body dementia because of the risk that neuroleptic sensitivity could worsen the motor symptoms. Some individuals with Lewy body dementia may benefit from the use of levodopa for their rigidity and loss of spontaneous movement.

There are three cholinesterase inhibitors currently available: donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne). While these drugs have been approved by the U.S. Food and Drug Administration to treat dementia in Alzheimer’s disease, only rivastigmine has been approved to treat Parkinson’s disease dementia (one of the Lewy body dementias). It is not clear if one of these medications is better for people with Lewy body dementia than the others. They all improve cognitive and behavioral symptoms and usually do not significantly increase symptoms of parkinsonism ¹². However, in one study of Parkinson’s disease dementia, a disorder very similar to dementia with Lewy bodies, rivastigmine was associated with a mild increase in tremor in some patients. These medications can have some side effects (most commonly gastrointestinal upset) and may not help behavioral problems in all people with Lewy body dementia. However, these medications can be a good first choice to treat behavioral symptoms ¹². It is important to note that the use of cholinesterase inhibitors is a long-term treatment strategy for behavioral symptoms and benefits are not immediately observed.

Cholinesterase inhibitors do not always help behavioral symptoms. And sometimes reassurance and distraction do not work. What options are available for people with Lewy body dementia who still have hallucinations or are aggressive? The answer depends on the type and severity of behavioral symptoms.

If a person’s hallucinations are not disturbing, or if they are not in danger harming themselves or others, then it may be better to not treat them. However, if the hallucinations are very disturbing or if there is a fear that the person may hurt themselves or others, then additional help is needed.

The newer or atypical antipsychotics may be helpful for people with Lewy body dementia if used conservatively. Atypical antipsychotics include:

• aripiprazole (Abilify)
• clozapine (Clozaril)
• fluoxetine & olanzapine (Symbyax)
• iloperidone (Fanapt)
• olanzapine (Zyprexa)
• paliperidone (Invega)
• quetiapine (Seroquel)
• risperidone (Risperdal)
• ziprasidone (Geodon).

Many Lewy body dementia experts prefer quetiapine or clozapine to control difficult behavioral symptoms ¹².

Clozapine requires frequent blood tests to monitor for the development of potential blood problems. Both quetiepine and clozapine should be given at the lowest dose possible and for the shortest length of time possible to control symptoms.

Some atypical antipsychotic medications (like olanzapine and risperidone) should be avoided if possible, because they have higher incidences of side effects, such as increased parkinsonism, sedation, and orthostatic hypotension. Ask your loved one’s physician about possible side effects on motor function before he or she prescribes an atypical antipsychotic medication.

Caregivers should note that several years ago the U.S. Food and Drug Administration (FDA) issued a warning that all antipsychotic medications, both atypical and typical, increase the risk of death in elderly patients with dementia (this includes people with LBD). Doctors and families should discuss the risks associated with antipsychotic medication use, including the side effects of the medication, against the risks of potential physical harm and distress to the patient or caregivers as a result of behavioral symptoms. Caregivers should contact the doctor about any side effects they observe that do not go away within a few days.

Cognitive Symptoms

Medications called cholinesterase inhibitors are considered the standard treatment for cognitive symptoms in Lewy body dementia. These medications were developed to treat Alzheimer’s disease. However, some researchers believe that people with Lewy body dementia may be even more responsive to these types of medications than those with Alzheimer’s disease.

Movement Symptoms

Movement symptoms may be treated with a Parkinson’s disease medication called levodopa, but if the symptoms are mild, it may be best to not treat them in order to avoid potential medication side-effects.

Visual Hallucinations

If hallucinations are disruptive or upsetting, your physician may recommend a cautious trial of a newer antipsychotic medication. (Please see WARNING below). Of note, the dementia medications called cholinesterase inhibitors have also been shown to be effective in treating hallucinations and other psychiatric symptoms of Lewy body dementia.

WARNING:
Up to 50% of patients with Lewy body dementia who are treated with any antipsychotic medication may experience severe neuroleptic sensitivity, such as worsening cognition, heavy sedation, increased or possibly irreversible parkinsonism, or symptoms resembling neuroleptic malignant syndrome (NMS), which can be fatal. (NMS causes severe fever, muscle rigidity and breakdown that can lead to kidney failure.)

REM Sleep Behavior Disorder (RBD)

RBD can be quite responsive to treatment, so your physician may recommend a medication like melatonin and/or clonazepam.

Neuroleptic Sensitivity

Severe sensitivity to neuroleptics is common in Lewy body dementia. Neuroleptics, also known as antipsychotics, are medications used to treat hallucinations or other serious mental disorders. While traditional antipsychotic medications (e.g. haloperidol) are commonly prescribed for individuals with Alzheimer’s with disruptive behavior, these medications can affect the brain of an individual with Lewy body dementia differently, sometimes causing severe side effects (see below). For this reason, traditional antipsychotic medications like haloperidol should be avoided. Some newer ‘atypical’ antipsychotic medications like risperidone may also be problematic for someone with Lewy body dementia. Quetiapine is preferred by some Lewy body dementia experts. If quetiapine is not tolerated or is not helpful, clozapine should be considered, but requires ongoing blood tests to assure a rare but serious blood condition does not develop. Hallucinations must be treated very conservatively, using the lowest doses possible under careful observation for side effects.

Medication Side Effects

Speak with your doctor about possible side effects. The following drugs may cause sedation, motor impairment or confusion:

• Benzodiazepines, tranquilizers like diazepam and lorazepam
• Anticholinergics (antispasmodics), such as oxybutynin and glycopyrrolate
• Some surgical anesthetics
• Older antidepressants
• Certain over-the-counter medications, including diphenhydramine and dimenhydrinate.
• Some medications, like anticholinergics, amantadine and dopamine agonists, which help relieve parkinsonian symptoms, might increase confusion, delusions or hallucinations.

NOTE: Be sure to meet with your anesthesiologist in advance of any surgery to discuss medication sensitivities and risks unique to Lewy body dementia. People with Lewy body dementia often respond to certain anesthetics and surgery with acute states of confusion or delirium and may have a sudden significant drop in functional abilities, which may or may not be permanent.

Possible alternatives to general anesthesia include a spinal or regional block. These methods are less likely to result in postoperative confusion. If you are told to stop taking all medications prior to surgery, consult with your doctor to develop a plan for careful withdrawal.

Non-Medical Treatments

Physical therapy options include cardiovascular, strengthening, and flexibility exercises, as well as gait training. Physicians may also recommend general physical fitness programs such as aerobic, strengthening, or water exercise.

Speech therapy may be helpful for low voice volume and poor enunciation. Speech therapy may also improve muscular strength and swallowing difficulties.

Occupational therapy may help maintain skills and promote function and independence. In addition to these forms of therapy and treatment, music and aroma therapy can also reduce anxiety and improve mood.

Individual and family psychotherapy can be useful for learning strategies to manage emotional and behavioral symptoms and to help make plans that address individual and family concerns about the future.

Support groups may be helpful for caregivers and persons with Lewy body dementia to identify practical solutions to day-to-day frustrations, and to obtain emotional support from others.

Prognosis Lewy body dementia

Like Alzheimer’s disease and Parkinson’s disease, Lewy body dementia is a neurodegenerative disorder that results in progressive intellectual and functional deterioration. There are no known therapies to stop or slow the progression of Lewy body dementia. Average survival after the time of diagnosis is similar to that in Alzheimer’s disease, about 8 years, with progressively increasing disability. It is possible, though, for the time span to be anywhere from 2 to 20 years, depending on several factors, including the person’s overall health, age and severity of symptoms.

Defining the stages of disease progression for Lewy body dementia is difficult. The symptoms, medicine management and duration of Lewy body dementia vary greatly from person to person. To further complicate the stages assessment, Lewy body dementia has a progressive but vacillating clinical course, and one of its defining symptoms is fluctuating levels of cognitive abilities, alertness and attention. Sudden decline is often caused by medications, infections or other compromises to the immune system and usually the person with Lewy body dementia returns to their baseline upon resolution of the problem. But for some individuals, it may also be due to the natural course of the disease.

Advice for People Living with Lewy Body Dementia

Coping with a diagnosis of Lewy body dementia and all that follows can be challenging. Getting support from family, friends, and professionals is critical to ensuring the best possible quality of life. Creating a safe environment and preparing for the future are important, too. Take time to focus on your strengths, enjoy each day, and make the most of your time with family and friends. Here are some ways to live with Lewy body dementia day to day.

Getting Help

Your family and close friends are likely aware of changes in your thinking, movement, or behavior. You may want to tell others about your diagnosis so they can better understand the reason for these changes and learn more about Lewy body dementia. For example, you could say that you have been diagnosed with a brain disorder called Lewy body dementia, which can affect thinking, movement, and behavior. You can say that you will need more help over time. By sharing your diagnosis with those closest to you, you can build a support team to help you manage Lewy body dementia.

As Lewy body dementia progresses, you will likely have more trouble managing everyday tasks such as taking medication, paying bills, and driving. You will gradually need more assistance from family members, friends, and perhaps professional caregivers. Although you may be reluctant to get help, try to let others partner with you so you can manage responsibilities together. Remember, Lewy body dementia affects your loved ones, too. You can help reduce their stress when you accept their assistance.

Finding someone you can talk with about your diagnosis—a trusted friend or family member, a mental health professional, or a spiritual advisor—may be helpful.

Consider Safety

The changes in thinking and movement that occur with Lewy body dementia require attention to safety issues. Consider these steps:

• Fill out and carry the Lewy body dementia Medical Alert Wallet Card and present it any time you are hospitalized, require emergency medical care, or meet with your doctors. It contains important information about medication sensitivities.
• Consider subscribing to a medical alert service, in which you push a button on a bracelet or necklace to access an emergency phone line if you need emergency help.
• Address safety issues in your home, including areas of fall risk, poor lighting, stairs, or cluttered walkways. Think about home modifications that may be needed, such as installing grab bars in the bathroom or modifying stairs with ramps. Ask your doctor to refer you to a home health agency for a home safety evaluation.
• Talk with your doctor about Lewy body dementia and driving, and have your driving skills evaluated, if needed.

Plan for Your Future

There are many ways to plan ahead. Here are some things to consider:

• If you are working, consult with a legal and financial expert about planning for disability leave or retirement. Symptoms of Lewy body dementia will interfere with work performance over time, and it is essential to plan now to obtain benefits you are entitled to.
• Consult with an attorney who specializes in elder law or estate planning to help you write or update important documents, such as a living will, healthcare power of attorney, and will.
• Identify local resources for home care, meals, and other services before you need them so you know whom to call when the time comes.
• Explore moving to a retirement or continuing care community where activities and varying levels of care can be provided over time, as needed. Ask about staff members’ experience caring for people with Lewy body dementia.

Find Enjoyment Every Day

It is important to focus on living with Lewy body dementia. Your attitude can help you find enjoyment in daily life. Despite the many challenges and adjustments, you can have moments of humor, tenderness, and gratitude with the people closest to you.

Make a list of events and activities you can still enjoy—then find a way to do them! For example, listening to music, exercising, or going out for a meal allows you to enjoy time with family and friends. If you can’t find pleasure in daily life, consult your doctor or another healthcare professional to discuss effective ways to cope and move forward. Let your family know if you are struggling emotionally so they can offer support.

Treatment and Management of Lewy Body Dementia

While Lewy body dementia currently cannot be prevented or cured, some symptoms may respond to treatment for a period of time ¹³. A comprehensive treatment plan may involve medications, physical and other types of therapy, and counseling. Changes to make the home safer, equipment to make everyday tasks easier, and social support are also very important.

A skilled care team often can provide suggestions to help improve quality of life for both people with Lewy body dementia and their caregivers.

Building a Care Team

After receiving a diagnosis, a person with Lewy body dementia may benefit from seeing a neurologist who specializes in dementia and/or movement disorders. A good place to find an Lewy body dementia specialist is at a dementia or movement disorders clinic in an academic medical center in your community. If such a specialist cannot be found, a general neurologist should be part of the care team. Ask a primary care physician for a referral.

Depending on an individual’s particular symptoms, other professionals may also be helpful:

• Physical therapists can help with movement problems through cardiovascular, strengthening, and flexibility exercises, as well as gait training and general physical fitness programs.
• Speech therapists may help with low voice volume, voice projection, and swallowing difficulties.
• Occupational therapists help identify ways to more easily carry out everyday activities, such as eating and bathing, to promote independence.
• Music or expressive arts therapists may provide meaningful activities that can reduce anxiety and improve well-being.
• Mental health counselors can help people with Lewy body dementia and their families learn how to manage difficult emotions and behaviors and plan for the future.
• Palliative care specialists can help improve a person’s quality of life by relieving disease symptoms at any stage of illness.

Support groups are another valuable resource for both people with Lewy body dementia and caregivers. Sharing experiences and tips with others in the same situation can help people identify practical solutions to day-to-day challenges and get emotional and social support.

Medications

Several drugs and other treatments are available to treat Lewy body dementia symptoms. It is important to work with a knowledgeable health professional because certain medications can make some symptoms worse. Some symptoms can improve with nondrug treatments.

Cognitive Symptoms

Some medications used to treat Alzheimer’s disease also may be used to treat the cognitive symptoms of Lewy body dementia. These drugs, called cholinesterase inhibitors, act on a chemical in the brain that is important for memory and thinking. They may also improve behavioral symptoms.

The U.S. Food and Drug Administration (FDA) approves specific drugs for certain uses after rigorous testing and review. The FDA has approved one Alzheimer’s drug, rivastigmine (Exelon®), to treat cognitive symptoms in Parkinson’s disease dementia. This and other Alzheimer’s drugs can have side effects such as nausea and diarrhea.

Movement Symptoms

Lewy body dementia-related movement symptoms may be treated with a Parkinson’s medication called carbidopa-levodopa (Sinemet®, Parcopa®, Stalevo®). This drug can help improve functioning by making it easier to walk, get out of bed, and move around. However, it cannot stop or reverse the progress of the disease.

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. If prescribed, carbidopa-levodopa usually begins at a low dose and is increased gradually. Other Parkinson’s medications are less commonly used in people with Lewy body dementia due to a higher frequency of side effects.

A surgical procedure called deep brain stimulation, which can be very effective in treating the movement symptoms of Parkinson’s disease, is not recommended for people with Lewy body dementia because it can result in greater cognitive impairment.

People with Lewy body dementia may benefit from physical therapy and exercise. Talk with your doctor about what physical activities are best.

Sleep Disorders

Sleep problems may increase confusion and behavioral problems in people with Lewy body dementia 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 Lewy body dementia symptom, involves acting out one’s dreams, leading to lost sleep and even injuries to sleep partners. Clonazepam (Klonopin®), 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 Lewy body dementia. If it is severe, a sleep specialist may prescribe a stimulant to help the person stay awake during the day.

Some people with Lewy body dementia may have difficulty falling asleep. If trouble sleeping at night (insomnia) persists, a physician may recommend a prescription medication to promote sleep. It is important to note that treating insomnia and other sleep problems in people with Lewy body dementia has not been extensively studied, and that treatments may worsen daytime sleepiness and should be used with caution.

Certain sleep problems can be addressed without medications. Increasing daytime exercise or activities and avoiding lengthy or frequent naps can promote better sleep. Avoiding alcohol, caffeine, or chocolate late in the day can help, too. Some over-the-counter medications can also affect sleep, so review all medications and supplements with a physician.

Behavioral and Mood Problems

Behavioral and mood problems in people with Lewy body dementia can arise from hallucinations or delusions. They may also be a result of pain, illness, stress or anxiety, and the inability to express frustration, fear, or feeling overwhelmed. The person may resist care or lash out verbally or physically.

Caregivers must try to be patient and use a variety of strategies to handle such challenging behaviors. Some behavioral problems can be managed by making changes in the person’s environment and/or treating medical conditions. Other problems may require medication.

The first step is to visit a doctor to see if a medical condition unrelated to Lewy body dementia is causing the problem. Injuries, fever, urinary tract or pulmonary infections, pressure ulcers (bed sores), and constipation can worsen behavioral problems. Increased confusion can also occur.

Certain medications used to treat Lewy body dementia symptoms or other diseases may also cause behavioral problems. For example, some sleep aids, pain medications, bladder control medications, and drugs used to treat Lewy body dementia-related movement symptoms can cause confusion, agitation, hallucinations, and delusions. Similarly, some anti-anxiety medicines can actually increase anxiety in people with Lewy body dementia. Review your medications with your doctor to determine if any changes are needed.

Not all behavioral problems are caused by illness or medication. A person’s surroundings—including levels of stimulation or stress, lighting, daily routines, and relationships—can lead to behavior issues. Caregivers can alter the home environment to try to minimize anxiety and stress for the person with Lewy body dementia. In general, people with Lewy body dementia benefit from having simple tasks, consistent schedules, regular exercise, and adequate sleep. Large crowds or overly stimulating environments can increase confusion and anxiety.

Hallucinations and delusions are among the biggest challenges for Lewy body dementia caregivers. The person with Lewy body dementia may not understand or accept that the hallucinations are not real and become agitated or anxious. Caregivers can help by responding to the fears expressed instead of arguing or responding factually to comments that may not be true. By tuning in to the person’s emotions, caregivers can offer empathy and concern, maintain the person’s dignity, and limit further tension.

Cholinesterase inhibitors may reduce hallucinations and other psychiatric symptoms of Lewy body dementia. These medications may have side effects, such as nausea, and are not always effective. However, they can be a good first choice to treat behavioral symptoms. Cholinesterase inhibitors do not affect behavior immediately, so they should be considered part of a long-term strategy.

Antidepressants can be used to treat depression and anxiety, which are common in Lewy body dementia. Two types of antidepressants, called selective serotonin reuptake inhibitors and norepinephrine reuptake inhibitors, are often well tolerated by people with Lewy body dementia.

In some cases, antipsychotic medications are necessary to treat Lewy body dementia-related behavioral symptoms to improve both the quality of life and safety of the person with Lewy body dementia and his or her caregiver. These types of medications must be used with caution because they can cause severe side effects and can worsen movement symptoms.

If antipsychotics are prescribed, it is very important to use the newer kind, called atypical antipsychotics. These medications should be used at the lowest dose possible and for the shortest time possible to control symptoms. Many Lewy body dementia experts prefer quetiapine (Seroquel®) or clozapine (Clozaril®, FazaClo®) to control difficult behavioral symptoms.

Typical (or traditional) antipsychotics, such as haloperidol (Haldol®), generally should not be prescribed for people with Lewy body dementia. They can cause dangerous side effects.

Warning About Antipsychotics

People with Lewy body dementia may have severe reactions to or side effects from antipsychotics, medications used to treat delusions, hallucinations, or agitation. These side effects include increased confusion, worsened parkinsonism, extreme sleepiness, and low blood pressure that can result in fainting (orthostatic hypotension). Caregivers should contact the doctor if these side effects continue after a few days.

Some antipsychotics, including olanzapine (Zyprexa®) and risperidone (Risperdal®), should be avoided, if possible, because they are more likely than others to cause serious side effects.

In rare cases, a potentially deadly condition called neuroleptic malignant syndrome can occur. Symptoms of this condition include high fever, muscle rigidity, and muscle tissue breakdown that can lead to kidney failure. Report these symptoms to your doctor immediately.

Antipsychotic medications increase the risk of death in elderly people with dementia, including those with Lewy body dementia. Doctors, patients, and family members must weigh the risks of antipsychotic use against the risks of physical harm and distress that may occur as a result of untreated behavioral symptoms.

Other Treatment Considerations

Lewy body dementia affects the part of the nervous system that regulates automatic actions like blood pressure and digestion. One common symptom is orthostatic hypotension, low blood pressure that can cause dizziness and fainting. Simple measures such as leg elevation, elastic stockings, and, when recommended by a doctor, increasing salt and fluid intake can help. If these measures are not enough, a doctor may prescribe medication.

Urinary incontinence (loss of bladder control) should be treated cautiously because certain medications used to treat this condition may worsen cognition or increase confusion. Consider seeing a urologist. Constipation can usually be treated by exercise and changes in diet, though laxatives and stool softeners may be necessary.

People with Lewy body dementia are often sensitive to prescription and over-the-counter medications for other medical conditions. Talk with your doctor about any side effects seen in a person with Lewy body dementia.

If surgery is planned and the person with Lewy body dementia is told to stop taking all medications beforehand, ask the doctor to consult the person’s neurologist in developing a plan for careful withdrawal. In addition, be sure to talk with the anesthesiologist in advance to discuss medication sensitivities and risks unique to Lewy body dementia. People with Lewy body dementia who receive certain anesthetics may become confused or delirious and have a sudden, significant decline in functional abilities, which may become permanent.

Depending on the procedure, possible alternatives to general anesthesia may include a spinal or regional block. These methods are less likely to result in confusion after surgery. Caregivers should also discuss the use of strong pain relievers after surgery, since people with Lewy body dementia can become delirious if these drugs are used too freely.

Vitamins and Supplements

The use of vitamins and supplements to treat Lewy body dementia symptoms has not been studied extensively and is not recommended as part of standard treatment. Vitamins and supplements can be dangerous when taken with other medicines. People with Lewy body dementia should tell their doctors about every medication they take. Be sure to list prescription and over-the-counter medicines, as well as vitamins and supplements.

1. National Institutes of Health. National Institute on Aging. What Is Lewy Body Dementia ? https://www.nia.nih.gov/health/what-lewy-body-dementia[↩][↩][↩][↩][↩][↩]
2. Genetic and Rare Diseases Information Center. Lewy body dementia. https://rarediseases.info.nih.gov/diseases/3243/lewy-body-dementia[↩]
3. Lewy Body Dementia Association. 10 Things You Should Know about LBD. https://www.lbda.org/content/10-things-you-should-know-about-lbd[↩][↩][↩][↩]
4. The National Institute of Neurological Disorders and Stroke. Dementia With Lewy Bodies Information Page. https://www.ninds.nih.gov/Disorders/All-Disorders/Dementia-Lewy-Bodies-Information-Page[↩]
5. NINDS Dementia With Lewy Bodies Information Page. National Institute of Neurological Disorders and Stroke (NINDS). November 2015; http://www.ninds.nih.gov/disorders/dementiawithlewybodies/dementiawithlewybodies.htm.[↩]
6. Howard A Crystal, MD. Dementia With Lewy Bodies. Medscape Reference. April 2014; http://emedicine.medscape.com/article/1135041-overview#a4.[↩]
7. U.S. National Library of Medicine. MedlinePlus. Lewy Body Disease. https://medlineplus.gov/lewybodydisease.html[↩]
8. National Institutes of Health. National Institute on Aging. What Causes Lewy Body Dementia ? https://www.nia.nih.gov/health/what-causes-lewy-body-dementia[↩][↩]
9. National Institutes of Health. National Institute on Aging. Diagnosing Lewy Body Dementia. https://www.nia.nih.gov/health/diagnosing-lewy-body-dementia[↩][↩]
10. National Institutes of Health. National Institute on Aging. Symptoms of Lewy Body Dementia. https://www.nia.nih.gov/health/symptoms-lewy-body-dementia[↩][↩]
11. Lewy Body Dementia Association, DLB and PDD Diagnostic Criteria. http://www.lbda.org/node/470[↩]
12. Lewy Body Dementia Association. Treatment of Behavioral Symptoms: When to Consider Antipsychotic Medications in LBD. https://www.lbda.org/content/treatment-behavioral-symptoms-when-consider-antipsychotic-medications-lbd[↩][↩][↩]
13. National Institutes of Health. National Institute on Aging. Treatment and Management of Lewy Body Dementia. https://www.nia.nih.gov/health/treatment-and-management-lewy-body-dementia[↩]