Dementia with Lewy Bodies

What Is Dementia with Lewy Bodies?

Dementia with Lewy bodies (DLB) is a progressive brain disorder that causes problems with thinking, movement, sleep, mood, and behavior. “Progressive” means symptoms get worse over time. It is one of the two types of Lewy body dementia (LBD); the other is Parkinson’s disease dementia.

DLB accounts for 15 percent of all cases of dementia in the United States, making it the second most common type, after Alzheimer’s disease. Over one million people in the United States are diagnosed with dementia with Lewy bodies.

Causes of Dementia With Lewy Bodies

Lewy bodies are abnormal clumps of a protein called alpha-synuclein that build up in and damage nerve cells in the brain. Symptoms gradually worsen over time as more Lewy bodies accumulate in the brain.

Researchers don’t fully understand what causes Lewy bodies to form or how they destroy nerve cells in the brain. The current thought is that a combination of genetics, environmental risk factors, and the natural aging process all interact to cause DLB to develop.

Certain variants of the APOE and GBA genes increase a person's likelihood of developing dementia with Lewy bodies. However, these genetic variants don’t guarantee that a person will develop DLB.

Symptoms of Dementia With Lewy Bodies

DLB symptoms usually start in people older than 50, but cases in younger people have been reported. Symptoms vary and progress differently from person to person.

The most common symptoms of dementia with Lewy bodies affect movement, cognition (mental function), sleep, mood, behavior, and automatic (autonomic) processes such as heart rate, breathing, blood pressure, and body temperature regulation through sweating.

DLB Symptoms Affecting Movement

Dementia with Lewy bodies is a form of atypical parkinsonism. People with DLB often have symptoms that are similar to Parkinson’s disease, including:

• Slowed movements (bradykinesia)
• Stiffness (rigidity)
• Tremors

Other DLB symptoms affecting movement may include:

• Balance problems and frequent falling
• Decreased facial expression
• Difficulty swallowing (dysphagia)
• Loss of coordination
• Shuffling or freezing during walking
• Smaller handwriting than normal (micrographia)

DLB Symptoms Affecting Cognition

A common feature of dementia with Lewy bodies is mental function that changes from day to day or even within the same day. People with DLB switch from moments of alertness and attention to periods of confusion and unresponsiveness.

DLB symptoms affecting cognition may include:

• Inability to perform two or more tasks at once
• Memory loss (usually later on in the disease process)
• Poor judgment and problems with decision-making
• Problems with planning, reasoning, and problem-solving
• Trouble focusing or staying awake
• Trouble with depth perception and hand-eye coordination
• Visual hallucinations (seeing things that are not really there) affect about 80 percent of people with DLB and often begin in the earlier stages of the disease

DLB Symptoms Affecting Sleep

People with DLB commonly experience sleep disorders, including:

• Changes in sleep patterns
• Excessive daytime sleepiness or napping during the day
• Insomnia
• Rapid eye movement (REM) sleep behavior disorder
• Restless legs syndrome (RLS)
• Trouble telling dreams from reality after waking

DLB Symptoms Affecting Mood or Behavior

People with DLB may experience changes in mood or behavior, including:

• Anxiety
• Delusions (false beliefs or opinions that the person believes are true but are not in reality)
• Depression
• Lack of interest in events or daily activities
• Paranoia
• Restlessness, irritability, agitation, or aggression
• Social withdrawal

DLB Symptoms Affecting Autonomic Function

Dysautonomia refers to problems affecting the autonomic nervous system (ANS), which controls processes in the body that are not under voluntary control. These processes include blood pressure, breathing, heart rate, digestion, and regulating internal body temperature.

Dysautonomia symptoms of dementia with Lewy bodies may include:

• Constipation
• Dizziness or fainting spells
• Increased sensitivity to heat and cold
• Large drops in blood pressure, especially with changes in position (orthostatic hypotension)
• Problems with bowel or bladder control (incontinence)
• Sexual dysfunction
• Trouble regulating body temperature

Dementia With Lewy Bodies Diagnosis

There are no medical tests for diagnosing dementia with Lewy bodies. Similarities with other conditions—Parkinson’s disease and Alzheimer’s disease—can make accurate diagnosis of dementia with Lewy bodies more challenging.

The criteria for an DLB diagnosis include a progressive decline in cognitive ability, plus two or more of the following symptoms:

• Fluctuating alertness and cognition
• Parkinson-like symptoms
• REM sleep behavior disorder
• Visual hallucinations

Several specialists may be involved in forming a "probable diagnosis”, including neurologists, who care for disorders of the brain and nervous system, and neuropsychologists, who study how disorders of the brain affect brain function and cognition.

Your neurologist will ask about your personal and family medical histories, current medications, and how your symptoms affect your daily life. They will perform a physical examination, including assessing your cognition, movement, reflexes, sensations, strength, and coordination.

Your neurologist may conduct further tests to support the diagnosis of dementia with Lewy bodies if there are atypical features. These tests are typically not needed to make a diagnosis.

• Imaging studies including magnetic resonance imaging (MRI), positron emission tomography (PET), and single photon emission computed tomography (SPECT)
• Blood tests
• Neuropsychological examination
• Sleep studies

Dementia With Lewy Bodies Treatment

There is no cure for DLB. Treatments may help manage symptoms and improve your quality of life.

Medications

Medications can help reduce or control symptoms affecting your cognition, mood, movement, and sleep. Such medications may include:

• Cholinesterase inhibitors: Medications such as donepezil, galantamine, and rivastigmine increase levels of neurotransmitters in the brain that are important for alertness, thought, and memory. They ease cognitive problems experienced by some people with DLB.
• Anti-depressants: Medications such as selective serotonin reuptake inhibitors (SSRIs) are used to treat depression and mood swings.
• Carbidopa-levodopa: This drug works by increasing the levels of dopamine in the brain. It may help reduce Parkinson’s-like symptoms in DLB, such as slowed movements, stiffness, or tremors.
• Clonazepam and melatonin: These medications treat REM sleep behavior disorder to improve sleep quality.
• Memantine: This medication decreases the chemical messenger glutamate in the brain. It specifically treats moderate-to-severe dementia.
• Pimavanserin: This medication is used to treat hallucinations and delusions for people with Parkinson’s disease dementia. It may be helpful for people with DLB with these symptoms.

Rehabilitative Therapies

Rehabilitative therapies can help improve your quality of life, manage symptoms, and reduce complications. Your neurologist may recommend:

• Occupational therapy: Occupational therapists can help you find new and easier ways to perform daily tasks.
• Physical therapy: Physical therapists teach you exercises to improve balance, reduce fall risk, and keep you active.
• Psychotherapy: Psychotherapists can help you manage anxiety and depression that often accompany DLB. They may prescribe anti-depressant or anti-anxiety medications.
• Speech therapy: Speech therapists can improve your ability to communicate clearly and swallow and eat more effectively.

Palliative Care Support

Palliative care focuses on quality of life and symptom relief for anyone at any stage of a long-term illness.

At Penn Medicine, our palliative care specialists provide physical, mental, emotional, and spiritual support for people with dementia with Lewy bodies and their caregivers.

Learn more about Palliative Care at Penn

Care for Dementia With Lewy Bodies at Penn Medicine

The Lewy Body Dementia Association recognizes Penn Medicine’s Movement Disorders Center as one of 22 Research Centers of Excellence. This designation recognizes our commitment to DLB research and to providing the most advanced diagnostic and treatment options for people with DLB.

As the leading movement disorders center in the Philadelphia region, we provide comprehensive medical, rehabilitative, and psychological care for thousands of patients with diseases like Lewy body dementia, including both Parkinson’s disease dementia and dementia with Lewy bodies, each year.

Make an Appointment

Please call 800-789-7366 or make an appointment.

Penn Programs & Services for Dementia with Lewy Bodies