The Dartmouth Memory Handbook

Section 4: Other Memory Disorders

 

 

Vascular Dementia and

Vascular Cognitive Impairment

 

From the National Institute on Aging
Alzheimer’s Disease Education and Referral Center

https://www.nia.nih.gov/alzheimers/publication/dementias/types-dementia#mid
Last Updated: July 29, 2016
 
Vascular dementia and vascular cognitive impairment (VCI) are caused by injuries to the
vessels supplying blood to the brain. These disorders can be caused by brain damage
from multiple strokes or any injury to the small vessels carrying blood to the brain.
Dementia risk can be significant even when individuals have suffered only small strokes.
Vascular dementia and VCI arise as a result of risk factors that similarly increase the risk
for cerebrovascular disease (stroke), including atrial fibrillation,
hypertension, diabetes, and high cholesterol. Vascular dementia also has been
associated with a condition called amyloid angiopathy, in which amyloid plaques
accumulate in the blood-vessel walls, causing them to break down and rupture.
Symptoms of vascular dementia and VCI can begin suddenly and progress or subside
during one’s lifetime.

 

Some types of vascular dementia include:

 
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and
Leukoencephalopathy (CADASIL)
This inherited form of cardiovascular disease results in a thickening of the walls of smalland
medium-sized blood vessels, eventually stemming the flow of blood to the brain. It is
associated with mutations of a specific gene called Notch3, which gives instructions to a
protein on the surface of the smooth muscle cells that surround blood vessels. CADASIL
is associated with multi-infarct dementia, stroke, migraine with aura (migraine preceded
by visual symptoms), and mood disorders. The first symptoms can appear in people
between ages 20 and 40. Many people with CADASIL are undiagnosed. People with
first-degree relatives who have CADASIL can be tested for genetic mutations to the
Notch3 gene to determine their own risk of developing CADASIL.
 
Multi-Infarct Dementia
This type of dementia occurs when a person has had many small strokes that damage
brain cells. One side of the body may be disproportionally affected, and multi-infarct
dementia may impair language or other functions, depending on the region of the brain
that is affected. Doctors call these “local” or “focal” symptoms, as opposed to the “global”
symptoms seen in AD that tend to affect several functions and both sides of the body.
When the strokes occur on both sides of the brain, however, dementia is more likely
than when stroke occurs on one side of the brain. In some cases, a single stroke can
damage the brain enough to cause dementia. This so-called single-infarct dementia is
more common when stroke affects the left side of the brain—where speech centers are
located—and/or when it involves the hippocampus, the part of the brain that is vital for
memory.
 
Subcortical Vascular Dementia
This is also called Binswanger’s disease. This is a rare form of dementia that involves
extensive microscopic damage to the small blood vessels and nerve fibers that make up
white matter, the “network” part of the brain believed to be critical for relaying messages
between regions. The symptoms of Binswanger’s are related to the disruption of
subcortical neural circuits involving short-term memory, organization, mood, attention,
decision-making, and appropriate behavior. A characteristic feature of this disease is
psychomotor slowness, such as an increase in the time it takes for a person to think of a
letter and then write it on a piece of paper.
 
Other symptoms include urinary incontinence that is unrelated to a urinary tract
condition, trouble walking, clumsiness, slowness, lack of facial expression, and speech
difficulties. Symptoms tend to begin after age 60, and they progress in a stepwise
manner. People with subcortical vascular disease often have high blood pressure, a
history of stroke, or evidence of disease of the large blood vessels in the neck or heart
valves. Treatment is aimed at preventing additional strokes and may include drugs to
control blood pressure.
 
Mixed Dementia
Autopsy studies looking at the brains of people who had dementia suggest that a
majority of those age 80 and older probably had “mixed dementia,” caused by both ADrelated
neurodegenerative processes and vascular disease-related processes. In fact,
some studies indicate that mixed vascular-degenerative dementia is the most common
cause of dementia in the elderly. In a person with mixed dementia, it may not be clear
exactly how many of a person’s symptoms are due to AD or another type of dementia. In
one study, approximately 40 percent of people who were thought to have AD were found
after autopsy to also have some form of cerebrovascular disease. Several studies have
found that many of the major risk factors for vascular disease also may be risk factors for
AD.
 
Researchers are still working to understand how underlying disease processes in mixed
dementia influence each other. It is not clear, for example, if symptoms are likely to be
worse when a person has brain changes reflecting multiple types of dementia. Nor do
we know if a person with multiple dementias can benefit from treating one type, for
example, when a person with AD controls high blood pressure and other vascular
disease risk factors.
 
Lewy Body Dementia
 

From the National Institute on Aging

Alzheimer’s Disease Education and Referral Center

https://www.nia.nih.gov/alzheimers/publication/lewy-body-dementia/introduction
Last updated September, 2015
 
Introduction
Lewy body dementia (LBD) is a complex and challenging brain disorder. It is complex
because it affects many parts of the brain in ways that scientists are trying to understand
more fully. It is challenging because its many possible symptoms make it hard to do
everyday tasks that once came easily.
 
Although less known than its “cousins” Alzheimer’s disease and Parkinson’s disease,
LBD is not a rare disorder. More than 1 million Americans, most of them older adults, are
affected by its disabling changes in the ability to think and move.
As researchers seek better ways to treat LBD—and ultimately to find a cure—people
with LBD and their families struggle day to day to get an accurate diagnosis, find the
best treatment, and manage at home.
 
The Basics of Lewy Body Dementia
LBD is a disease associated with abnormal deposits of a protein called alpha-synuclein
in the brain. These deposits, called Lewy bodies, affect chemicals in the brain whose
changes, in turn, can lead to problems with thinking, movement, behavior, and mood.
LBD is one of the most common causes of dementia, after Alzheimer’s
disease and vascular disease.
 
Dementia is a severe loss of thinking abilities that interferes with a person’s capacity to
perform daily activities such as household tasks, personal care, and handling finances.
Dementia has many possible causes, including stroke, brain tumor, depression, and
vitamin deficiency, as well as disorders such as LBD, Parkinson’s, and Alzheimer’s.
Diagnosing LBD can be challenging for a number of reasons. Early LBD symptoms are
often confused with similar symptoms found in other brain diseases like Alzheimer’s.
Also, LBD can occur alone or along with Alzheimer’s or Parkinson’s disease.
There are two types of LBD—dementia with Lewy bodies and 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.
 
Who Is Affected by LBD?
LBD affects more than 1 million individuals in the United States. LBD typically begins at
age 50 or older, although younger people occasionally will develop it. LBD appears to
affect slightly more men than women.
 
LBD 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. 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 LBD, usually before a diagnosis is made, symptoms can be mild,
and people can function fairly normally. As the disease advances, people with LBD
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 LBD symptoms may respond to treatment for a period of time. Currently, there is
no cure for the disease. 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.
 
Understanding Terms
The terms used to describe Lewy body dementia (LBD) can be confusing. Doctors and
researchers may use different terms to describe the same condition. In this chapter, the
term Lewy body dementia is used to describe all dementias whose primary cause is
abnormal deposits of Lewy bodies in the brain.
The two types of LBD are:
  • Dementia with Lewy bodies, in which cognitive (thinking) symptoms appear
    within a year of movement problems
  • Parkinson’s disease dementia, in which cognitive symptoms develop more
    than a year after the onset of movement problems
As LBD progresses, symptoms of both types of LBD are very similar.
 
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. These abnormal deposits are now called “Lewy
bodies.”
 
Lewy bodies are made of a protein called alpha-synuclein. In the healthy brain, alphasynuclein
plays a number of important roles in neurons (nerve cells) in the brain,
especially at synapses, where brain cells communicate with each other. In LBD, alphasynuclein
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 LBD:
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)
 
Types of Lewy Body Dementia
Lewy body dementia includes two related conditions—dementia with Lewy bodies and
Parkinson’s disease dementia. The difference between them lies largely in the timing of
cognitive (thinking) and movement symptoms. In dementia with Lewy bodies, cognitive
symptoms are noted within a year of parkinsonism, any condition that involves the types
of movement changes, such as tremor or muscle stiffness, seen in Parkinson’s disease.
In Parkinson’s disease dementia, movement symptoms are pronounced in the early
stages, with cognitive symptoms developing years later.
 
Dementia with Lewy Bodies
People with dementia with Lewy bodies have a decline in thinking ability that may look
somewhat like Alzheimer’s disease. But over time they also develop movement and
other distinctive symptoms that suggest dementia with Lewy bodies.
 
Symptoms that distinguish this form of dementia from others may include:
  • Visual hallucinations early in the course of dementia
  • Fluctuations in cognitive ability, attention, and alertness
  • Slowness of movement, difficulty walking, or rigidity (parkinsonism)
  • Sensitivity to medications used to treat hallucinations
  • REM sleep behavior disorder, in which people physically act out their dreams by
    yelling, flailing, punching bed partners, and falling out of bed
  • More trouble with complex mental activities, such as multitasking, problem
    solving, and analytical thinking, than with memory
 
Parkinson’s Disease Dementia
This type of LBD starts as a movement disorder, with symptoms such as slowed
movement, muscle stiffness, tremor, and a shuffling walk. These symptoms are
consistent with a diagnosis of Parkinson’s disease. Later on, cognitive symptoms of
dementia and changes in mood and behavior may arise.
 
Not all people with Parkinson’s develop dementia, and it is difficult to predict who will.
Being diagnosed with Parkinson’s late in life is a risk factor for Parkinson’s disease
dementia.
 
Causes and Risk Factors
Risk factors for LBD include age and disorders such as Parkinson's disease.
The precise cause of LBD is unknown, but scientists are learning more about its biology
and genetics. 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.
Scientists are also learning about risk factors for LBD. Age is considered the greatest
risk factor. Most people who develop the disorder are over age 50.
Other known risk factors for LBD 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 LBD.
 
Genetics—While having a family member with LBD may increase a person’s
risk, LBD 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 LBD. 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
LBD. 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.
 
Common Symptoms
People with LBD may not have every LBD symptom, and the severity of symptoms can
vary greatly from person to person. 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 include changes in cognition, movement, sleep, and
behavior.
 
Cognitive Symptoms
LBD 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 LBD 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 LBD
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 LBD 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 LBD 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 LBD, 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.
 
Movement Symptoms
Some people with LBD 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:
  • Movement problems
  • 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 LBD 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 LBD 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 LBD is
    aware of these symptoms. REM sleep behavior disorder appears in some people
    years before other LBD 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 LBD. 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 LBD
    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 LBD Symptoms
People with LBD 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
  • Changes in blood pressure
  • Dizziness
  • Fainting
  • Frequent falls
  • Sensitivity to heat and cold
  • Sexual dysfunction
  • Urinary incontinence
  • Constipation
  • A poor sense of smell
Diagnosis
An accurate diagnosis helps people with Lewy body dementia get the right medical
treatment. It’s important to know which type of LBD 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 LBD 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.
 
Regardless of the initial symptoms, over time people with LBD 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.
 
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 LBD earlier and more
accurately as researchers identify which symptoms help distinguish it from similar
disorders.
 
Difficult as it is, getting an accurate diagnosis of LBD 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
While a diagnosis of LBD 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.
 
Who Can Diagnose LBD?
Many physicians and other medical professionals are not familiar with LBD, so patients
may consult several doctors before receiving a diagnosis. Visiting a family doctor is often
the first step for people who are experiencing changes in thinking, movement, or
behavior. However, neurologists—doctors who specialize in disorders of the brain and
nervous system—generally have the expertise needed to diagnose LBD. Geriatric
psychiatrists, neuropsychologists, and geriatricians may also be skilled in diagnosing the
condition.
 
If a specialist cannot be found in your community, ask the neurology department of the
nearest medical school for a referral. A hospital affiliated with a medical school may also
have a dementia or movement disorders clinic that provides expert evaluation. See
the Resources section.
 
Tests Used to Diagnose LBD
Doctors perform physical and neurological examinations and various tests to distinguish
LBD from other illnesses. An evaluation may include:
  • Medical history and examination - A review of previous and current illnesses,
    medications, and current symptoms and tests of movement and memory give the
    doctor valuable information.
     
  • Magnetic resonance image of the brain.
     
  • Medical tests - Laboratory studies can help rule out other diseases and hormonal
    or vitamin deficiencies that can be associated with cognitive changes.
     
  • Brain imaging - Computed tomography or magnetic resonance imaging can
    detect brain shrinkage or structural abnormalities and help rule out other possible
    causes of dementia or movement symptoms.
     
  • Neuropsychological Tests - These tests are used to assess memory and other
    cognitive functions and can help identify affected brain regions.
 
There are no brain scans or medical tests that can definitively diagnose LBD. Currently,
LBD can be diagnosed with certainty only by a brain autopsy after death.
 
However, researchers are studying ways to diagnose LBD 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.
 
Other Helpful Information
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 LBD 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.
 
Treatment and Management
While LBD currently cannot be prevented or cured, some symptoms may respond to
treatment for a period of time. 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 LBD and their caregivers.
 
Building a Care Team
After receiving a diagnosis, a person with LBD may benefit from seeing a neurologist
who specializes in dementia and/or movement disorders. A good place to find an LBD
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.
 
A doctor can work with other types of healthcare providers. Depending on an individual’s
particular symptoms, other professionals may 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 wellbeing.
     
  • Mental health counselors can help people with LBD and their families learn
    how to manage difficult emotions and behaviors and plan for the future.
     
  • Support groups are another valuable resource for both people with LBD 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. To
    find a support group near you, see the Resources section.
 
Medications
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 symptoms can improve with nondrug treatments.
 
Medications For Cognitive Symptoms
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 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.
 
Medications For Movement Symptoms
LBD-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 LBD 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 LBD because it can result in greater cognitive impairment.
 
People with LBD may benefit from physical therapy and exercise. Talk with your doctor
about what physical activities are best.
 
Sleep and Dementia
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 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 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 LBD 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-thecounter
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 LBD 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. Making changes in the person’s environment and/or treating
medical conditions can manage some behavioral problems. Other problems may require
medication.
 
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.
 
Increased confusion can also occur.
 
Certain medications used to treat LBD symptoms or other diseases may also cause
behavioral problems. For example, some sleep aids, pain medications, bladder control
medications, and drugs used to treat LBD-related movement symptoms can cause
confusion, agitation, hallucinations, and delusions. Similarly, some anti-anxiety
medicines can actually increase anxiety in people with LBD. 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 LBD. In general, people with
LBD benefit from having simple tasks, consistent schedules, regular exercise, and
adequate sleep. Large crowds or overly stimulating environments can increase
confusion and anxiety.
 
Coping with Behavioral Problems
Follow these steps in consultation with a physician to address behavioral problems:
Rule out physical causes, like infection, pain, or other medical conditions.
Review current prescription and over-the-counter medications.
Look for environmental or social factors that contribute to behavioral problems.
Consider treating with medications if necessary and watch for side effects.
 
Hallucinations and delusions are among the biggest challenges for LBD caregivers. The
person with LBD 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
LBD. 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
LBD. Two types of antidepressants, called selective serotonin reuptake inhibitors and
norepinephrine reuptake inhibitors, are often well tolerated by people with LBD.
In some cases, antipsychotic medications are necessary to treat LBD-related behavioral
symptoms to improve both the quality of life and safety of the person with LBD 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 LBD experts prefer quetiapine or
clozapine to control difficult behavioral symptoms.
 
Typical (or traditional) antipsychotics, such as haloperidol (Haldol¨), generally
should not be prescribed for people with LBD. They can cause dangerous side effects.
 
Warning About Antipsychotics
People with LBD 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.
 
Antipsychotics 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 LBD. 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
LBD 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.
Low blood pressure can make a person with LBD feel dizzy.
 
People with LBD 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 LBD.
 
If surgery is planned and the person with LBD 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 LBD. People with LBD 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 LBD can become delirious if these drugs are used too freely.
 
Vitamins and Supplements
The use of vitamins and supplements to treat LBD 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 LBD
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.
 
Advice for People Living with Lewy Body Dementia
Get help from family and friends as needed to manage LBD day to day.
Coping with a diagnosis of LBD 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 LBD 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 LBD. 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 LBD.
 
As LBD 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, LBD 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. See
the Resources section to find supportive services in your area.
 
Consider Safety
The changes in thinking and movement that occur with LBD require attention to safety
issues. Consider these steps:
 
  • Fill out and carry the LBD 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 911 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 LBD 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 LBD 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 LBD.
 
Find Enjoyment Every Day
A positive attitude can help people with LBD adjust to a new life.
It is important to focus on living with LBD. 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.
 
Caring for a Person with Lewy Body Dementia
As someone who is caring for a person with LBD, you will take on many different
responsibilities over time. You do not have to face these responsibilities alone. Many
sources of help are available, from adult day centers and respite care to online and inperson
support groups.
 
Below are some important actions you can take to adjust to your new roles, be realistic
about your situation, and care for yourself. See the Resources section for more
information.
 
Educate Others About LBD
Most people, including many healthcare professionals, are not familiar with LBD. In
particular, emergency room physicians and other hospital workers may not know that
people with LBD are extremely sensitive to antipsychotic medications. Caregivers can
educate healthcare professionals and others by:
 
  • Informing hospital staff of the LBD diagnosis and medication sensitivities, and
    requesting that the person’s neurologist be consulted before giving any drugs to
    control behavior problems.
  • Sharing educational pamphlets and other materials with doctors, nurses, and
    other healthcare professionals who care for the person with LBD. Materials are
    available from the Lewy Body Dementia Association (see the “Resources”
    section).
  • Teaching family and friends about LBD so they can better understand your
    situation.
 
Prepare for Emergencies
People with LBD may experience sudden declines in functioning or unpredictable
behaviors that can result in visits to the emergency room. Infections, pain, or other
medical conditions often cause increased confusion or behavioral problems. Caregivers
can prepare for emergencies by having available:
  • A list of the person’s medications and dosages
  • A list of the person’s health conditions, including allergies to medicines or
  • Copies of health insurance card(s)
  • Copies of healthcare advance directives, such as a living will
  • Contact information for doctors, family members, and friends
 
Adjust Expectations
You will likely experience a wide range of emotions as you care for the person with LBD.
Sometimes, caregiving will feel loving and rewarding. Other times, it will lead to anger,
impatience, resentment, or fatigue. You must recognize your strengths and limitations,
especially in light of your past relationship with the person. Roles may change between a
husband and wife or between a parent and adult children. Adjusting expectations can
allow you to approach your new roles realistically and to seek help as needed.
People approach challenges at varied paces. Some people want to learn everything
possible and be prepared for every scenario, while others manage best by taking one
day at a time. Caring for someone with LBD requires a balance. On one hand, you
should plan for the future. On the other hand, you may want to make each day count in
personal ways and focus on creating enjoyable and meaningful moments.
 
Care for Yourself
As a caregiver, you play an essential role in the life of the person with LBD, so it is
critical for you to maintain your own health and wellbeing. You may be at increased risk
for poor sleep, depression, or illness as a result of your responsibilities. Watch for signs
of physical or emotional fatigue such as irritability, withdrawal from friends and family,
and changes in appetite or weight.
 
Address Family Concerns
Not all family members may understand or accept LBD at the same time, and this can
create conflict. Some adult children may deny that parents have a problem, while others
may be supportive. It can take a while to learn new roles and responsibilities.
Family members who visit occasionally may not see the symptoms that primary
caregivers see daily and may underestimate or minimize your responsibilities or stress.
Professional counselors can help with family meetings or provide guidance on how
families can work together to manage LBD.
 
Helping Children and Teens Cope with LBD
Children need help to cope with a relative's Lewy body dementia.
When someone has Lewy body dementia, it affects the whole family, including children
and grandchildren. Children notice when something “doesn’t seem right.” Telling them in
age-appropriate language that someone they know or love has been diagnosed with a
brain disorder can help them make sense of the changes they see. Give them enough
information to answer questions or provide explanations without overwhelming them.
Children and teens may feel a loss of connection with the person with LBD who has
problems with attention or alertness. They may also resent the loss of a parent
caregiver’s attention and may need special time with him or her. Look for signs of stress
in children, such as poor grades at school, withdrawal from friendships, or unhealthy
behaviors at home. Parents may want to notify teachers or counselors of the LBD
diagnosis in the family so they can watch for changes in the young person that warrant
attention.
 
Here are some other ways parents can help children and teens adjust to a family
member with LBD:
  • People with LBD and young relatives can find ways to spend time together. Help
    them keep up with normal activities such as sports, clubs, and other hobbies
    outside the home. Suggest ways for kids to engage with the relative with LBD
    through structured activities or play. For example, the child or teen can make a
    cup of tea for the person with LBD.
     
  • Find online resources for older children and teens so they can learn about
    dementia and LBD. See the “Resources” section for more information.
It is important for families to make time for fun. Many challenges can be faced when they
are balanced with enjoyable times. While LBD creates significant changes in family
routines, children and teens will cope more effectively if the disorder becomes part of,
but not all of, their lives.
 
Research—The Road Ahead
Lewy body dementia research helps move us toward better diagnosis, treatment, and
even prevention.
 
There is a great deal to learn about LBD. At a basic level, why does alpha-synuclein
accumulate into Lewy bodies, and how do Lewy bodies cause the symptoms of LBD? It
is also of increasing interest to the Alzheimer’s and Parkinson’s disease research
communities. LBD represents an important link between these other brain disorders, and
research into one disease often contributes to better understanding of the others.
Many avenues of research focus on improving our understanding of LBD. Some
researchers are working to identify the specific differences in the brain between
dementia with Lewy bodies and Parkinson’s disease dementia. Others are looking at the
disease’s underlying biology, genetics, and environmental risk factors. Still other
scientists are trying to identify biomarkers (biological indicators of disease), improve
screening tests to aid diagnosis, and research new treatments.
 
Scientists hope that new knowledge about LBD will one day lead to more
effective treatments and even ways to cure and prevent the disorder. Until then,
researchers need volunteers with and without LBD for clinical studies.
 
To find out more about clinical trials, talk with a doctor or visit the National Institutes of
Health Web site at: www.clinicaltrials.gov and www.nih.gov/health/clinicaltrials.
 
Resources
 
Federal Government:
 
National Institute on Aging
Alzheimer’s Disease Education and Referral (ADEAR) Center
1-800-438-4380 (toll-free)
adear@nia.nih.gov
www.nia.nih.gov/alzheimers
 
National Institute of Neurological Disorders and Stroke
1-800-352-9424 (toll-free)
www.ninds.nih.gov
 
MedlinePlus
National Library of Medicine
www.medlineplus.gov
www.medlineplus.gov/spanish
 
General Information:
 
Lewy Body Dementia Association
1-800-539-9767 (toll-free LBD Caregiver Link)
1-404-935-6444 (national office)
www.lbda.org
 
Michael J. Fox Foundation for Parkinson’s Research
1-800-708-7644 (toll-free)
www.michaeljfox.org
 
National Parkinson Foundation
1-800-473-4636 (toll-free helpline)
contact@parkinson.org
www.parkinson.org
 
Parkinson’s Disease Foundation
1-800-457-6676 (toll-free helpline)
info@pdf.org
www.pdf.org
 
Support Services and Resources
 
Family Caregiver Alliance
The Alliance provides information and referral, education, and other services to
caregivers of people with chronic, disabling health conditions.
1-800-445-8106 (toll-free)
info@caregiver.org
www.caregiver.org
 
For Children and Teens
Although the following Alzheimer’s organizations are not specific to Lewy body
dementia, their dementia-related resources and networks may be helpful for children and
teens.
 
ADEAR Center Resources for Children and Teens
www.nia.nih.gov/alzheimers/resources-children-and-teens-about-alzheimers
 
Alzheimer’s Association Kids & Teens
1-800-272-3900 (toll-free)
www.alz.org/living_with_alzheimers_just_for_kids_and_teens.asp
 
Alzheimer’s Foundation of America (AFA) Teens
1-866-232-8484 (toll-free)
www.afateens.org
 
Diagnosis, Treatment, and Research:
 
The Lewy Body Dementia Association (LBDA) maintains a list of its scientific and
medical advisors. Many of these physicians see patients at their clinics or may make
referrals to other specialists in your area. See www.lbda.org/content/lbda-scientificadvisory-
council-sac. For general tips on finding a doctor, visit
www.lbda.org/content/finding-doctor-diagnose-and-treat-lbd.
 
The Alzheimer’s Disease Education and Referral Center maintains a list of research
centers funded by the National Institute on Aging at NIH where you may find medical
specialists in Lewy body dementia: www.nia.nih.gov/alzheimers/alzheimers-diseaseresearch-
centers.
 
Acknowledgments
The National Institute on Aging and the National Institute of Neurological Disorders and
Stroke thank the following people for their contributions to the vision and creation of this
booklet:
 
Lisa Snyder, MSW, LCSW
Director, Quality of Life Programs
Shiley-Marcos Alzheimer’s Disease Research Center
University of California, San Diego
 
Christina Gigliotti, PhD
Community Health Program Supervisor
Shiley-Marcos Alzheimer’s Disease Research Center
University of California, San Diego
 
Angela Taylor
Director of Programs
Lewy Body Dementia Association
 
Scientific and Medical Reviewers from the LBDA Scientific Advisory
Council
 
Douglas Galasko, MD
Co-Director, Shiley-Marcos Alzheimer’s Disease Research Center
University of California, San Diego
 
Howard Hurtig, MD
Chair, Department of Neurology
Pennsylvania Hospital
 
James Leverenz, MD
Director, Cleveland Lou Ruvo Center for Brain Health–Neurological Institute
 
Caregiver Reviewers
Nancy Archibald
Kathleen Burr
Paul Smith
Joy Walker
 
Frontotemporal Disorders
Information for Patients, Families, and Caregivers
 

From the National Institute on Aging

Alzheimer’s Disease Education and Referral Center

https://www.nia.nih.gov/alzheimers/publication/frontotemporal-disorders/introduction
Last Updated: January 22, 2015
 
Introduction
Few people have heard of frontotemporal disorders, which lead to dementias that affect
personality, behavior, language, and movement. These disorders are little known outside
the circles of researchers, clinicians, patients, and caregivers who study and live with
them. Although frontotemporal disorders remain puzzling in many ways, researchers are
finding new clues that will help them solve this medical mystery and better understand
other common dementias.
 
The symptoms of frontotemporal disorders gradually rob people of basic abilities—
thinking, talking, walking, and socializing— that most of us take for granted. They often
strike people in the prime of life, when they are working and raising families. Families
suffer, too, as they struggle to cope with the person’s daily needs as well as changes in
relationships and responsibilities.
 
The Basics of Frontotemporal Disorders
Frontotemporal disorders are the result of damage to neurons (nerve cells) in parts of
the brain called the frontal and temporal lobes. As neurons die in the frontal and
temporal regions, these lobes atrophy, or shrink. Gradually, this damage causes
difficulties in thinking and behaviors normally controlled by these parts of the brain. Many
possible symptoms can result, including unusual behaviors, emotional problems, trouble
communicating, difficulty with work, or difficulty with walking.
 
A Form of Dementia
Frontotemporal disorders are forms of dementia caused by a family of brain diseases
known as frontotemporal lobar degeneration (FTLD). Dementia is a severe loss of
thinking abilities that interferes with a person’s ability to perform daily activities such as
working, driving, and preparing meals. Other brain diseases that can cause dementia
include Alzheimer’s disease and multiple strokes. Scientists estimate that FTLD may
cause up to 10 percent of all cases of dementia and may be about as common as
Alzheimer’s among people younger than age 65. Roughly 60 percent of people with
FTLD are 45 to 64 years old. People can live with frontotemporal disorders for up to 10
years, sometimes longer, but it is difficult to predict the time course for an individual
patient. The disorders are progressive, meaning symptoms get worse over time. In the
early stages, people may have just one type of symptom. As the disease progresses,
other types of symptoms appear as more parts of the brain are affected.
No cure or treatments that slow or stop the progression of frontotemporal disorders are
available today. However, research is improving awareness and understanding of these
challenging conditions. This progress is opening doors to better diagnosis, improved
care, and, eventually, new treatments.
 
FTD? FTLD? Understanding Terms
One of the challenges shared by patients, families, clinicians, and researchers is
confusion about how to classify and label frontotemporal disorders. A diagnosis by one
doctor may be called something else by a second, and the same condition or syndrome
referred to by another name by a pathologist who examines the brain after death.
For many years, scientists and physicians used the term frontotemporal dementia (FTD)
to describe this group of illnesses. After further research, FTD is now understood to be
just one of several possible variations and is more precisely called behavioral variant
frontotemporal dementia, or bvFTD.
 
This chapter uses the term frontotemporal disorders to refer to changes in behavior and
thinking that are caused by underlying brain diseases collectively called frontotemporal
lobar degeneration (FTLD). FTLD is not a single brain disease but rather a family of
neurodegenerative diseases, any one of which can cause a frontotemporal
disorder. Frontotemporal disorders are diagnosed by physicians and psychologists
based on a person’s symptoms and results of brain scans and genetic tests. With the
exception of known genetic causes, FTLD can be identified definitively only by brain
autopsy after death.
 
Frontotemporal disorders affect the frontal and temporal lobes of the brain. They can
begin in the frontal lobe, the temporal lobe, or both. Initially, frontotemporal disorders
leave other brain regions untouched, including those that control short-term memory.
The frontal lobes, situated above the eyes and behind the forehead both on the right and
left sides of the brain, direct executive functioning. This includes planning and
sequencing (thinking through which steps come first, second, third, and so on),
prioritizing (doing more important activities first and less important activities last),
multitasking (shifting from one activity to another as needed), and monitoring and
correcting errors.
 
When functioning well, the frontal lobes also help manage emotional responses. They
enable people to avoid inappropriate social behaviors, such as shouting loudly in a
library or at a funeral. They help people make decisions that make sense for a given
situation. When the frontal lobes are damaged, people may focus on insignificant details
and ignore important aspects of a situation or engage in purposeless activities. The
frontal lobes are also involved in language, particularly linking words to form sentences,
and in motor functions, such as moving the arms, legs, and mouth.
 
The temporal lobes, located below and to the side of each frontal lobe on the right and
left sides of the brain, contain essential areas for memory but also play a major role in
language and emotions. They help people understand words, speak, read, write, and
connect words with their meanings. They allow people to recognize objects and to relate
appropriate emotions to objects and events. When the temporal lobes are dysfunctional,
people may have difficulty recognizing emotions and responding appropriately to them.
 
Which lobe—and part of the lobe—is affected first determines which symptoms appear
first. For example, if the disease starts in the part of the frontal lobe responsible for
decision-making, then the first symptom might be difficulty managing finances. If it
begins in the part of the temporal lobe that connects emotions to objects, then the first
symptom might be an inability to recognize potentially dangerous objects—a person
might reach for a snake or plunge a hand into boiling water, for example.
 
Types of Frontotemporal Disorders
Frontotemporal disorders can be grouped into three types, defined by the earliest
symptoms physicians identify when they examine patients.
• Progressive behavior/personality decline—characterized by changes in
personality, behavior, emotions, and judgment (called behavioral variant
frontotemporal dementia).
• Progressive language decline—marked by early changes in language ability,
including speaking, understanding, reading, and writing (called primary
progressive aphasia).
• Progressive motor decline—characterized by various difficulties with physical
movement, including the use of one or more limbs, shaking, difficulty walking,
frequent falls, and poor coordination (called corticobasal syndrome, supranuclear
palsy, or amyotrophic lateral sclerosis).
 
In the early stages it can be hard to know which of these disorders a person has
because symptoms and the order in which they appear can vary widely from one person
to the next. Also, the same symptoms can appear in different disorders. For example,
language problems are most typical of primary progressive aphasia but can also appear
later in the course of behavioral variant frontotemporal dementia. The table below
summarizes the three types of frontotemporal disorders and lists the various terms that
could be used when clinicians diagnose these disorders.
 
Behavioral Variant Frontotemporal Dementia
The most common frontotemporal disorder, behavioral variant frontotemporal
dementia (bvFTD), involves changes in personality, behavior, and judgment. People with
this dementia can act strangely around other people, resulting in embarrassing social
situations. Often, they don’t know or care that their behavior is unusual and don’t show
any consideration for the feelings of others. Over time, language and/or movement
problems may occur, and the person needs more care and supervision.
 
In the past, bvFTD was called Pick's disease, named after Arnold Pick, the German
scientist who first described it in 1892. The term Pick’s disease is now used to describe
abnormal collections in the brain of the protein tau, called "Pick bodies." Some patients
with bvFTD have Pick bodies in the brain, and some do not.
 
Primary Progressive Aphasia
(see also p.109)
 
Primary progressive aphasia (PPA) involves changes in the ability to communicate—to
use language to speak, read, write, and understand what others are saying. Problems
with memory, reasoning, and judgment are not apparent at first but can develop over
 
time. In addition, some people with PPA may experience significant behavioral changes,
similar to those seen in bvFTD, as the disease progresses.
 
There are three types of PPA, categorized by the kind of language problems seen at
first. Researchers do not fully understand the biological basis of the different types of
PPA. But they hope one day to link specific language problems with the abnormalities in
the brain that cause them.
 
In semantic PPA, also called semantic dementia, a person slowly loses the ability to
understand single words and sometimes to recognize the faces of familiar people and
common objects.
 
In agrammatic PPA, also called progressive nonfluent aphasia, a person has more and
more trouble producing speech. Eventually, the person may no longer be able to speak
at all. He or she may eventually develop movement symptoms similar to those seen in
corticobasal syndrome.
 
In logopenic PPA, a person has trouble finding the right words during conversation but
can understand words and sentences. The person does not have problems with
grammar.
 
Movement Disorders
Two rare neurological disorders associated with FTLD, corticobasal syndrome (CBS)
and progressive supranuclear palsy (PSP), occur when the parts of the brain that control
movement are affected. The disorders may affect thinking and language abilities, too.
CBS can be caused by corticobasal degeneration—gradual atrophy and loss of nerve
cells in specific parts of the brain. This degeneration causes progressive loss of the
ability to control movement, typically beginning around age 60. The most prominent
symptom may be the inability to use the hands or arms to perform a movement despite
normal strength (called apraxia). Symptoms may appear first on one side of the body,
but eventually both sides are affected. Occasionally, a person with CBS first has
language problems or trouble orienting objects in space and later develops movement
symptoms.
 
PSP causes problems with balance and walking. People with the disorder typically move
slowly, experience unexplained falls, lose facial expression, and have body stiffness,
especially in the neck and upper body—symptoms similar to those of Parkinson’s
disease. A hallmark sign of PSP is trouble with eye movements, particularly looking
down. These symptoms may give the face a fixed stare. Behavior problems can also
develop.
 
Other movement-related frontotemporal disorders include frontotemporal dementia with
parkinsonism and frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS)
Frontotemporal dementia with parkinsonism can be an inherited disease caused by a
genetic tau mutation. Symptoms include movement problems similar to those of
Parkinson’s disease, such as slowed movement, stiffness, and balance problems, and
changes in behavior or language.
 
FTD-ALS is a combination of bvFTD and ALS, commonly called Lou Gehrig’s disease.
Symptoms include the behavioral and/or language changes seen in bvFTD as well as
the progressive muscle weakness seen in ALS. Symptoms of either disease may appear
first, with other symptoms developing over time. Mutations in certain genes have been
found in some patients with FTD-ALS.
 
Causes
Frontotemporal lobar degeneration (FTLD) is not a single brain disease but rather a
family of brain diseases that share some common molecular features. Scientists are
beginning to understand the biological and genetic basis for the changes observed in
brain cells that lead to FTLD.
 
Scientists describe FTLD in terms of patterns of change in the brain seen in an autopsy
after death. These changes include loss of neurons and abnormal amounts or forms of
proteins called tau and TDP-43. These proteins occur naturally in the body and help
cells function properly. When the proteins don’t work properly, for reasons not yet fully
understood, neurons in specific brain regions are damaged.
 
In most cases, the cause of a frontotemporal disorder is unknown. In about 15 to 40
percent of people, a genetic (hereditary) cause can be identified. Individuals with a
family history of frontotemporal disorders are more likely to have a genetic form of the
disease than those without such a history.
 
Familial and inherited forms of frontotemporal disorders are often related to mutations
(permanent changes) in certain genes. Genes are basic units of heredity that tell cells
how to make the proteins the body needs to function. Even small changes in a gene may
produce an abnormal protein, which can lead to changes in the brain and, eventually,
disease.
 
Scientists have discovered several different genes that, when mutated, can lead to
frontotemporal disorders:
 
• Tau gene (also called the MAPT gene)—A mutation in this gene causes
abnormalities in a protein called tau, which forms tangles inside neurons and
ultimately leads to the destruction of brain cells. Inheriting a mutation in this gene
means a person will almost surely develop a frontotemporal disorder, usually
bvFTD, but the exact age of onset and symptoms cannot be predicted.
 
• PGRN gene—A mutation in this gene can lead to lower production of the protein
progranulin, which in turn causes TDP-43, a cellular protein, to go awry in brain
cells. Many frontotemporal disorders can result, though bvFTD is the most
common. The PGRN gene can cause different symptoms in different family
members and cause the disease to begin at different ages.
 
• VCP, CHMP2B, TARDBP, and FUS genes—Mutations in these genes lead to
very rare familial types of frontotemporal disorders.
 
• C9ORF72 gene—An unusual mutation in this gene appears to be the most
common genetic abnormality in familial frontotemporal disorders and familial
ALS. This mutation can cause a frontotemporal disorder, ALS, or both conditions
in a person.
 
Families affected by inherited and familial forms of frontotemporal disorders can help
scientists further research by participating in clinical studies and trials. For more
information, talk with a healthcare professional, contact any of the research centers
listed at the end of this booklet, or search www.clinicaltrials.gov.
 
Diagnosis
No single test, such as a blood test, can be used to diagnose a frontotemporal disorder.
A definitive diagnosis can be confirmed only by a genetic test in familial cases or a brain
autopsy after a person dies. To diagnose a probable frontotemporal disorder in a living
person, a doctor—usually a neurologist, psychiatrist, or psychologist—will:
 
Record a person’s symptoms, often with the help of family members or friends
Compile a personal and family medical history
 
Perform a physical exam and order blood tests to help rule out other similar
conditions
 
If appropriate, order testing to uncover genetic mutations
Conduct a neuropsychological evaluation to assess behavior, language, memory,
and other cognitive functions
 
Use brain imaging to look for changes in the frontal and temporal lobes.
 
Different types of brain imaging may be used. A magnetic resonance imaging (MRI)
scan shows changes in the size and shape of the brain, including the frontal and
temporal lobes. It may reveal other potentially treatable causes of the person’s
symptoms, such as a stroke or tumor. In the early stage of disease, the MRI may appear
normal. In this case, other types of imaging, such as positron emission tomography
(PET) or single photon emission computed tomography (SPECT), may be useful. PET
and SPECT scans measure activity in the brain by monitoring blood flow, glucose usage,
and oxygen usage. Other PET scans can help rule out a diagnosis of Alzheimer's.
Frontotemporal disorders can be hard to diagnose because their symptoms—changes in
personality and behavior and difficulties with speech and movement—are similar to
those of other conditions. For example, bvFTD is sometimes misdiagnosed as a mood
disorder, such as depression, or as a stroke, especially when there are speech or
movement problems. To make matters more confusing, a person can have both a
frontotemporal disorder and another type of dementia such as Alzheimer's disease. Also,
since these disorders are rare, physicians may be unfamiliar with the relevant symptoms
and signs.
 
Getting the wrong diagnosis can be frustrating. Without knowing their true condition,
people with frontotemporal disorders may not get appropriate treatment to manage their
symptoms. Families may not get the help they need. People lose valuable time needed
to plan treatment and future care. The medical centers listed in Resources are places
where people with frontotemporal disorders can be diagnosed and treated.
 
Researchers are studying ways to diagnose frontotemporal disorders earlier and more
accurately. One area of research involves biomarkers, such as proteins or other
substances in the blood or cerebrospinal fluid, which can be used to measure the
progress of disease or the effects of treatment. Also being studied are ways to improve
brain imaging, including seeing the tau protein, and neuropsychological testing, which
assesses learning, language, problem solving, memory, and other thinking skills.
 
Common Symptoms
Symptoms of frontotemporal disorders vary from person to person and from one stage of
the disease to the next as different parts of the frontal and temporal lobes are affected.
In general, changes in the frontal lobe are associated with behavioral symptoms, while
changes in the temporal lobe lead to language and emotional disorders.
 
Symptoms are often misunderstood. Family members and friends may think that a
person is misbehaving, leading to anger and conflict. For example, a person with bvFTD
may neglect personal hygiene or start shoplifting. It is important to understand that
people with these disorders cannot control their behaviors and other symptoms.
Moreover, they lack any awareness of their illness, making it difficult to get help.
 
Behavioral Symptoms
 
• Problems with executive functioning—Problems with planning and
sequencing (thinking through which steps come first, second, third, and so on),
prioritizing (doing more important activities first and less important activities last),
multitasking (shifting from one activity to another as needed), and self-monitoring
and correcting behavior.
 
• Perseveration—A tendency to repeat the same activity or to say the same word
over and over, even when it no longer makes sense.
 
• Social disinhibition—Acting impulsively without considering how others
perceive the behavior. For example, a person might hum at a business meeting
or laugh at a funeral.
 
• Compulsive eating—Gorging on food, especially starchy foods like bread and
cookies, or taking food from other people’s plates.
 
• Utilization behavior—Difficulty resisting impulses to use or touch objects that
one can see and reach. For example, a person picks up the telephone receiver
while walking past it when the phone is not ringing and the person does not
intend to place a call.
 
Language Symptoms
 
• Aphasia—A language disorder in which the ability to use or understand words is
impaired but the physical ability to speak properly is normal.
 
• Dysarthria—A language disorder in which the physical ability to speak properly
is impaired (e.g., slurring) but the message is normal.
 
People with PPA may have only problems using and understanding words or also
problems with the physical ability to speak. People with both kinds of problems have
trouble speaking and writing. They may become mute, or unable to speak. Language
problems usually get worse, while other thinking and social skills may remain normal
longer before deteriorating.
 
Emotional Symptoms
 
• Apathy—A lack of interest, drive, or initiative. Apathy is often confused with
depression, but people with apathy may not be sad. They often have trouble
starting activities but can participate if others do the planning.
 
• Emotional changes—Emotions are flat, exaggerated, or improper. Emotions
may seem completely disconnected from a situation or are expressed at the
wrong times or in the wrong circumstances. For example, a person may laugh at
sad news.
 
• Social-interpersonal changes—Difficulty “reading” social signals, such as facial
expressions, and understanding personal relationships. People may lack
empathy—the ability to understand how others are feeling—making them seem
indifferent, uncaring, or selfish. For example, the person may show no emotional
reaction to illnesses or accidents that occur to family members.
 
Movement Symptoms
• Dystonia—Abnormal postures of body parts such as the hands or feet. A limb
may be bent stiffly or not used when performing activities that are normally done
with two hands.
 
• Gait disorder—Abnormalities in walking, such as walking with a shuffle,
sometimes with frequent falls.
 
• Tremor—Shakiness, usually of the hands.
 
• Clumsiness—Dropping of small objects or difficulty manipulating small items like
buttons or screws.
 
• Apraxia—Loss of ability to make common motions, such as combing one's hair
or using a knife and fork, despite normal strength.
 
• Neuromuscular weakness—Severe weakness, cramps, and rippling
movements in the muscles.
 
Treatment and Management
So far, there is no cure for frontotemporal disorders and no way to slow down or prevent
them. However, there are ways to manage symptoms. A team of specialists—doctors,
nurses, and speech, physical, and occupational therapists—familiar with these disorders
can help guide treatment.
 
Managing Behavior
The behaviors of a person with bvFTD can upset and frustrate family members and
other caregivers. It is natural to grieve for the “lost person,” but it is also important to
learn how to best live with the person he or she has become. Understanding changes in
personality and behavior and knowing how to respond can reduce caregivers’ frustration
and help them cope with the challenges of caring for a person with a frontotemporal
disorder.
 
Managing behavioral symptoms can involve several approaches. To ensure the safety of
a person and his or her family, caregivers may have to take on new responsibilities or
arrange care that was not needed before. For example, they may have to drive the
person to appointments and errands, care for young children, or arrange for help at
home.
 
It is helpful, though often difficult, to accept rather than challenge people with behavioral
symptoms. Arguing or reasoning with them will not help because they cannot control
their behaviors or even see that they are unusual or upsetting to others. Instead, be as
sensitive as possible and understand that it’s the illness “talking.” Frustrated caregivers
can take a “timeout”—take deep breaths, count to 10, or leave the room for a few
minutes.
 
To deal with apathy, limit choices and offer specific choices. Open-ended questions
(“What would you like to do today?”) are more difficult to answer than specific ones (“Do
you want to go to the movies or the shopping center today?”).
 
Maintaining the person’s schedule and modifying the environment can also help. A
regular schedule is less confusing and can help people sleep better. If compulsive eating
is an issue, caregivers may have to supervise eating, limit food choices, lock food
cabinets and the refrigerator, and distract the person with other activities. To deal with
other compulsive behaviors, caregivers may have to change schedules or offer new
activities.
 
Medications are available to treat certain behavioral symptoms. Antidepressants called
selective serotonin reuptake inhibitors are commonly prescribed to treat social
disinhibition and impulsive behavior. Patients with aggression or delusions sometimes
take low doses of antipsychotic medications. The use of Alzheimer’s disease
medications to improve behavioral and cognitive symptoms in people with bvFTD and
related disorders is being studied, though results so far have been mixed, with some
medications making symptoms worse. If a particular medication is not working, a doctor
may try another. Always consult a doctor before changing, adding, or stopping a drug.
 
Treating Language Problems
Treatment of primary progressive aphasia (PPA) has two goals—maintaining language
skills and using new tools and other ways to communicate. Treatment tailored to a
person’s specific language problem and stage of PPA generally works best. Since
language ability declines over time, different strategies may be needed as the illness
progresses.
 
To communicate without talking, a person with PPA may use a communication notebook
(an album of photos labeled with names of people and objects), gestures, and drawings.
Some people find it helpful to use or point to lists of words or phrases stored in a
computer or personal digital assistant.
 
Caregivers can also learn new ways of talking to someone with PPA. For example, they
can speak slowly and clearly, use simple sentences, wait for responses, and ask for
clarification if they don’t understand something.
 
A speech-language pathologist who knows about PPA can test a person’s language
skills and determine the best tools and strategies to use. Note that many speechlanguage
pathologists are trained to treat aphasia caused by stroke, which requires
different strategies from those used with PPA. (See the Resources section to find
speech-language pathologists and other experts who know about frontotemporal
disorders.)
 
Managing Movement Problems
No treatment can slow down or stop frontotemporal-related movement disorders, though
medications and physical and occupational therapy may provide modest relief.
For people with corticobasal syndrome (CBS), movement difficulties are sometimes
treated with medications for Parkinson’s disease. But these medicines offer only minimal
or temporary improvement. Physical and occupational therapy may help people with
CBS move more easily. Speech therapy may help them manage language symptoms.
For people with progressive supranuclear palsy (PSP), sometimes Parkinson’s disease
drugs provide temporary relief for slowness, stiffness, and balance problems. Exercises
can keep the joints limber, and weighted walking aids—such as a walker with sandbags
over the lower front rung—can help maintain balance. Speech, vision, and swallowing
difficulties usually do not respond to any drug treatment. Antidepressants have shown
modest success. For people with abnormal eye movements, bifocals or special glasses
called prisms are sometimes prescribed.
 
People with FTD-ALS typically decline quickly over the course of 2 to 3 years. During
this time, physical therapy can help treat muscle symptoms, and a walker or wheelchair
may be useful. Speech therapy may help a person speak more clearly at first. Later on,
other ways of communicating, such as a speech synthesizer, can be used. The ALS
symptoms of the disorder ultimately make it impossible to stand, walk, eat, and breathe
on one’s own.
 
For any movement disorder caused by FTLD, a team of experts can help patients and
their families address difficult medical and caregiving issues. Physicians, nurses, social
workers, and physical, occupational, and speech therapists familiar with frontotemporal
disorders can ensure that people with movement disorders get appropriate medical
treatment and that their caregivers can help them live as well as possible.
 
The Future of Treatment
Researchers are continuing to explore the genetic and biological actions in the body that
lead to frontotemporal disorders. In particular, they seek more information about genetic
mutations that cause FTLD, as well as the disorders' natural history and disease
pathways. They also want to develop better ways, such as specialized brain imaging, to
track its progression, so that treatments, when they become available, can be directed to
the right people. The ultimate goal is to identify possible new drugs and other treatments
to test.
 
Researchers are also looking for better treatments for frontotemporal disorders. Possible
therapies that target the abnormal proteins found in the brain are being tested in the
laboratory and in animals. Clinical trials and studies are testing a number of possible
treatments in humans.
 
Clinical trials for individuals with frontotemporal disorders will require many participants.
People with frontotemporal disorders and healthy people may be able to take part. To
find out more about clinical trials, talk to your healthcare provider or
visit www.clinicaltrials.gov.
 
Caring for a Person with a Frontotemporal Disorder
In addition to managing the medical and day-to-day care of people with frontotemporal
disorders, caregivers can face a host of other challenges. These challenges may include
changing family relationships, loss of work, poor health, decisions about long-term care,
and end-of-life concerns.
 
Family Issues
People with frontotemporal disorders and their families often must cope with changing
relationships, especially as symptoms get worse. For example, the wife of a man with
bvFTD not only becomes her husband’s caregiver, but also takes on household
responsibilities he can no longer perform. Children may suffer the gradual “loss” of a
parent at a critical time in their lives. The symptoms of bvFTD often embarrass family
members and alienate friends. Life at home can become very stressful.
 
Work Issues
Frontotemporal disorders disrupt basic work skills, such as organizing, planning, and
following through on tasks. Activities that were easy before the illness began might take
much longer or become impossible. People lose their jobs because they can no longer
perform them. As a result, the caregiver might need to take a second job to make ends
meet—or reduce hours or even quit work to provide care and run the household. An
employment attorney can offer information and advice about employee benefits, family
leave, and disability if needed.
 
Workers diagnosed with any frontotemporal disorder can qualify quickly for Social
Security disability benefits through the “compassionate allowances” program. For more
information, see www.socialsecurity.gov/compassionateallowances or call 1-800-772-
1213.
 
Caregiver Health and Support
Caring for someone with a frontotemporal disorder can be very hard, both physically and
emotionally. To stay healthy, caregivers can do the following:
 
• Get regular health care.
 
• Ask family and friends for help with childcare, errands, and other tasks.
 
• Spend time doing enjoyable activities, away from the demands of caregiving.
Arrange for respite care—short-term caregiving services that give the regular
caregiver a break—or take the person to an adult day care center, a safe,
supervised environment for adults with dementia or other disabilities.
 
• Join a support group for caregivers of people with frontotemporal disorders. Such
groups allow caregivers to learn coping strategies and share feelings with others
in the same position.
 
Long-Term Care
For many caregivers, there comes a point when they can no longer take care of the
person with a frontotemporal disorder without help. The caregiving demands are simply
too great, perhaps requiring around-the-clock care. As the disease progresses,
caregivers may want to get home healthcare services or look for a residential care
facility, such as a group home, assisted living facility, or nursing home. The decision to
move the person with a frontotemporal disorder to a care facility can be difficult, but it
can also give caregivers peace of mind to know that the person is safe and getting good
care. The decreased level of stress may also improve the caregivers' relationship with
his or her loved one.
 
End-of-Life Concerns
People with frontotemporal disorders typically live 6 to 8 years with their conditions,
sometimes longer, sometimes less. Most people die of problems related to advanced
disease. For example, as movement skills decline, a person can have trouble
swallowing, leading to aspiration pneumonia, in which food or fluid gets into the lungs
and causes infection. People with balance problems may fall and seriously injure
themselves.
 
It is difficult, but important, to plan for the end of life. Legal documents, such as a will,
living will, and durable powers of attorney for health care and finances, should be
created or updated as soon as possible after a diagnosis of bvFTD, PPA, or a related
disorder. Early on, many people can understand and participate in legal decisions. But
as the illness progresses, it becomes harder to make such decisions.
 
A physician who knows about frontotemporal disorders can help determine the person’s
mental capacity. An attorney who specializes in elder law, disabilities, or estate planning
can provide legal advice, prepare documents, and make financial arrangements for the
caregiving spouse or partner and dependent children. If necessary, the person’s access
to finances can be reduced or eliminated.
 
Conclusion
It is impossible to predict the exact course of frontotemporal disorders. These disorders
are not easy to live with, but with help, people can meet the challenges and prepare for
the future. Getting an early, accurate diagnosis and the right medical team are crucial
first steps. Researchers and clinicians are working toward a deeper understanding of
frontotemporal disorders and better diagnosis and treatment to help people manage
these difficult conditions.
 
Primary Progressive Aphasia (PPA)
 

Northwestern University Feinberg School of Medicine

Cognitive Neurology and Alzheimer's Disease Center

(reprinted with permission)
http://brain.northwestern.edu/dementia/ppa/living.html
 
Primary progressive aphasia (PPA) is a form of cognitive impairment that involves a
progressive loss of language function. Language is a uniquely human faculty that allows
us to communicate with each other through the use of words. Our language functions
include speaking, understanding what others are saying, repeating things we have
heard, naming common objects, reading and writing. “Aphasia” is a general term used to
refer to deficits in language functions. PPA is caused by degeneration in the parts of the
brain that are responsible for speech and language.
 
PPA begins very gradually and initially is experienced as difficulty thinking of common
words while speaking or writing. PPA progressively worsens to the point where verbal
communication by any means is very difficult. The ability to understand what others are
saying or what is being read also declines. In the early stages, memory, reasoning and
visual perception are not affected by the disease and so individuals with PPA are able to
function normally in many routine daily living activities despite the aphasia. However, as
the illness progresses, other mental abilities also decline.
 
Adults of any age can develop PPA, but it is more common in people under the age of
65. People with PPA can have a variety of different language symptoms and no two
cases are exactly the same.
 
Symptoms
People with PPA can experience many different types of language symptoms.
In many instances, the person with PPA may be the first to note that something is wrong
and the complaints may initially be attributed to stress or anxiety. People with PPA
initially experience one or more of the following symptoms:
 
• Slowed or halting speech
 
• Decreased use of language
 
• Word-finding hesitations
 
• Sentences with abnormal word order in speech or e-mails
 
• Substitution of words (e.g., “table” instead of “chair”)
 
• Using words that are mispronounced or incomprehensible (e.g., “track” for
“truck”)
 
• Talking around a word (e.g., "We went to the place where you can get
bread")
 
• Difficulty understanding or following conversation despite normal hearing
 
• Sudden lapse in understanding simple words
 
• Forgetting the names of familiar objects
 
• Inability to think of names of people, even though the person is recognized
 
• Problems writing (e.g. difficulty writing checks or notes)
 
• Problems reading (e.g. difficulty following written directions or reading signs)
 
• New impairments in spelling
 
• Problems in arithmetic and calculations (e.g. making change, leaving a tip)
People with PPA tend to have similar clusters of symptoms. Researchers who specialize
in PPA currently recognize three subtypes: agrammatic, logopenic and semantic.
 
PPA-G (Agrammatic/Nonfluent Subtype): A problem with word-order and wordproduction
Speech is effortful and reduced in quantity. Sentences become gradually shorter and
word-finding hesitations become more frequent, occasionally giving the impression of
stammering or stuttering. Pronouns, conjunctions and articles are lost first. Word order
may be abnormal, especially in writing or e-mails. Words may be mispronounced or
used in the reverse sense (e.g., “he” for “she” or “yes” for “no”). Word understanding is
preserved but sentence comprehension may suffer if the sentences are long and
grammatically complex.
 
PPA-L (Logopenic Subtype): A problem with word-finding
In contrast to PPA-G, speech is fluent during causal small talk but breaks into
mispronunciations and word-finding pauses when a more difficult or precise word needs
to be used. Some people with PPA-L are very good at going around the word they
cannot find. They learn to use a less apt or simpler word as well as to insert fillers such
as “the thing that you use for it,” “you know what I mean,” or “whatchamacallit”. Spelling
errors are common. The naming of objects becomes impaired. Understanding long and
complex sentences can become challenging but the comprehension of single words is
preserved.
 
PPA-S (Semantic Subtype): A problem with word-understanding
The principal feature is a loss of word meaning, even of common words. When asked to
bring an orange, for example, the person may appear puzzled and may ask what an
“orange” means. Speech has very few nouns and is therefore somewhat empty of
meaning. However, it sounds perfectly fluent because of the liberal use of fillers. The
person may seem to have forgotten the names of familiar objects.
 
Causes
PPA arises when nerve cells in language-related parts of the brain malfunction. The
underlying diseases are called “degenerative” because they cause gradually progressive
nerve cell death that cannot be attributed to other causes such as head trauma,
infection, stroke or cancer. There are several types of neurodegeneration that can
cause PPA. The two most commonly encountered types are frontotemporal lobar
degeneration (FTLD) and Alzheimer’s disease (AD).
 
Both FTLD and AD can lead to many different patterns of clinical impairments,
depending on the region of the brain that bears the brunt of the nerve cell loss. When
AD or FTLD attacks the language areas (usually on the left side of the brain), PPA
results. PPA is caused by AD in approximately 30-40% of cases and by FTLD in
approximately 60-70% of cases. In contrast, PPA is a very rare manifestation of AD. In
the vast majority of patients with AD, the most prominent clinical symptom is a memory
loss for recent events (amnesia) rather than an impairment of language (aphasia). PPA
is therefore said to be an “atypical” consequence of AD. The logopenic type of PPA has
a particularly high probability of being caused by AD. Specialized positron emission
tomography (PET) scans and examination of the spinal fluid may help to resolve the
distinction between the two underlying diseases. Whether or not PPA is caused by AD
or FTLD can be determined definitively only at autopsy through examination of brain
tissue with a microscope.
 
This can be confusing because for reasons outlined in the previous paragraph, the word
“Alzheimer’s” can be used in two different ways. The term Alzheimer’s dementia (or
Dementia of the Alzheimer-Type) is used to designate a progressive loss of memory
leading to a more generalized loss of all cognitive functions. The term
Alzheimer’s disease (as opposed to Alzheimer’s dementia) is used in a different way to
designate a precise pattern of microscopic abnormalities in the brain. Sometimes these
abnormalities become concentrated in language areas (instead of memory areas) of the
brain and become the cause of PPA. So, while PPA patients don’t have
Alzheimer’s dementia, 30-40% may have an atypical form of Alzheimer’s disease. This
dual use of the word “Alzheimer’s” is confusing, even for the specialist, but is a feature of
medical nomenclature that is here to stay.
 
In the vast majority of individuals, PPA is not genetic. However, in a small number of
families, hereditary forms of FTLD can cause PPA. The most common gene implicated
in these families is the progranulin gene (GRN). Other, less common genes implicated in
FTLD include the microtubule associated protein tau (MAPT) and a newly discovered
gene, chromosome 9 open reading frame 72 (C9ORF72).
 
Even in families with genetic mutations, one family member may have PPA while others
may have behavioral variant frontotemporal degeneration (bvFTD) or movement
disorders, including corticobasal degeneration (CBD) or progressive supranuclear palsy
(PSP). In the presence of a genetic mutation, up to 50% of all family members will have
FTLD. Therefore, genetic testing is not usually recommended unless several family
members have clinical patterns characteristic of PPA, bvFTD, CBD or PSP. Before
proceeding with genetic testing, it’s necessary to meet with a genetic counselor to review
the implications of the results. The immediate purpose of genetic testing is to determine
whether the person has a mutation that is responsible for the disease. However, the
results have profound implications for family members who are healthy, especially those
of childbearing age. Do family members want to know the presence of a genetic disease
for which there is no treatment? Do they realize that a negative result does not rule out
the presence of a mutation in another gene not covered by the testing? Genetic testing
for clinical purposes is a serious step that should not be initiated lightly.
 
Progression
Because PPA is progressive, decline in language ability continues. Additionally, some
non-language abilities (memory, attention, judgment or changes in behavior and
personality) can be affected. Disinhibited, inappropriate behaviors (also seen in
behavioral variant frontotemporal degeneration) are more common with PPA-S while
impairments in problem-solving, multi-tasking movement and mobility (of the type seen
in CBD and PSP) are more common in PPA-G. The rate of decline is variable from
person to person and unfolds over many years. It is unclear why some people progress
more rapidly than others.
 
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Diagnosis of Primary Progressive Aphasia (PPA)
A thorough evaluation of PPA includes the following:
 
History:
First, a careful history is taken to establish that a condition of dementia exists. This often
requires that family members or friends be questioned about the patient’s behavior
because sometimes the patient is unaware of the symptoms (as in the case of memory
loss or personality changes) or may be unable to describe them due to aphasia.
 
Neurological Examination:
A neurological examination is done to determine if there are signs of dementia on a
simple screening of mental functions (the mental status examination) and also if there
are signs of motor or sensory symptoms that indicate other types of neurological
disorders might be causing the dementia. The neurologist will also order tests (e.g.,
blood tests, spinal tap, brain imaging studies) to further investigate the cause of the
symptoms.
 
Neuropsychological Examination:
A neuropsychological examination provides a more detailed evaluation of mental
functioning. This is especially important in the very early stages of illness when a routine
screening evaluation may not detect the problems the patient is experiencing. This
requires several hours and consists of paper-and-pencil or computer-administered tests
of mental abilities, including attention and concentration, language, learning and
memory, visual perception, reasoning and mood. The results can indicate if there are
abnormalities of thinking and behavior and also their degree--mild, moderate or severe.
It is often difficult to demonstrate that individuals with PPA have intact memory since we
usually test memory by telling a person some information and then asking them to
repeat it later on. In an individual with PPA, it may be impossible to repeat back the
information because of the aphasia. Therefore, it is important that testing is done
properly to make sure that there is not a true loss of memory.
 
Speech and Language Evaluation:
Since a decline in language abilities is the primary symptom of PPA, it is important to
determine which components of language use are most affected, how severely affected
they are, and what can be done to improve communication. A Speech-Language
Pathologist evaluates different aspects of language in detail and can make
recommendations for strategies to improve communication. Family members should be
included in the treatment sessions to educate them about how to facilitate
communication.
 
Psychosocial Evaluation:
PPA affects not only the individual who is suffering from this disorder, but also all people
who are close to the patient. The disorder has an impact on relationships, the ability to
continue working, the ability to perform many routine duties, and the ability to
communicate even the simplest of needs. Although there are many resources available
for individuals with memory loss, there are relatively fewer appropriate resources for
individuals with PPA, their relatives and friends. Evaluation with a social worker who is
familiar with PPA can address these issues and provide suggestions for dealing with
day-to-day frustrations and problems.
 
Brain Imaging Studies:
The evaluation for dementia also includes a brain imaging study. This is done in the form
of a computed axial tomography scan (CAT scan) or a magnetic resonance imaging
scan (MRI scan). Both of these methods provide a picture of the brain so that any
structural abnormalities, such as a stroke, tumor or hydrocephalus--all of which can give
rise to dementia-like symptoms, can be detected. In the case of degenerative brain
disease, the CAT scan and MRI scan may show “atrophy,” which suggests “shrinkage”
of the brain tissue. However, especially in early stages, they may not show anything. In
fact, the report often comes back “normal.” But this only means that there is no evidence
for a tumor or stroke. It cannot tell us anything about the microscopic degenerative
changes that have occurred.
 
Psychiatric Evaluation:
Sometimes there will also be a need for a psychiatric evaluation. This may be the case
when it is not clear if the changes in behavior are due to depression or another
psychiatric disturbance. Also, some individuals, especially those with PPA, may become
saddened by their condition and may require treatment for depression.
There are many thousands of people with PPA. Nonetheless, compared to the millions
of patients with Alzheimer-type amnestic dementias, PPA is rare. Furthermore, it can
start in a person’s 40s and 50s, an age range that physicians do not usually associate
with neurodegenerative diseases. Therefore, some people with PPA often see multiple
doctors and receive many different diagnoses before receiving the diagnosis of PPA.
 
Treatment:
There are no pills yet for PPA. Because of the 30%-40% probability of Alzheimer’s
disease (AD), some physicians will prescribe AD drugs such as Exelon (rivastigmine),
Razadyne (galantamine), Aricept (donepezil) or Namenda (memantine). None have
been shown to improve PPA. Medicine is also sometimes prescribed to manage
behavioral symptoms such as depression, anxiety, or agitation, which may occur later in
the course of the illness.
 
There are, however, life-enriching interventions and speech therapies that can help
improve a diagnosed person’s quality of life. The primary goal of treatment for language
impairments in individuals with PPA is to improve the ability to communicate. Because
the type of language problems experienced by patients with PPA may vary, the focus of
treatment for improving communication ability will also vary. A complete speech and
language evaluation provides the information needed to determine the type of treatment
that is most appropriate.
 
There are two basic approaches to speech therapy for PPA. One approach is to focus
treatment directly on the language skills that are impaired (for example, skills to enhance
word-retrieval abilities), and the other is to provide augmentative/alternative
communication strategies or devices. We recommend that both treatment approaches
be used in people with PPA. Regardless of which strategies are provided to people with
PPA, it is important that the family is involved in treatment and that the use of the
strategy in the natural environment is encouraged.
 
Suggestions for Effective Communication for Supportive Communication
Partners:
 
General
• Try not to interrupt and supply a word unless help is requested. Develop a cue
that implies help is needed.
 
• Don’t always correct or point out mistakes
 
• Ask for clarification or repetition, do not pretend to understand when you don’t.
 
• Simplify speech. Ask questions with two choices instead of open-ended
questions (e.g. “Do you want eggs or cereal?” instead of “What do you want for
breakfast?”).
 
• Have realistic expectations of the person’s communication strengths and areas of
difficulty.
 
Listening
• Listen for key pieces of information (who, what, where, when, why, how)
 
• Be aware of distracting background noise and reduce it if possible (e.g. turn off
TV, move to a quiet room)
 
Speaking
• Cue the speaker to talk around the word by encouraging them to tell you about
its purpose or function
 
• General category (e.g. fruit, clothing, sport)
 
• Physical description (e.g., large, blue, square)
 
• Location
 
• Synonym or antonym for the word
 
• Speak slowly and quietly
 
• Speak to the person as an adult; don’t talk down.
Writing
 
• Can be used when reading and writing are relatively well-preserved
 
• If the person with PPA has difficulty pronouncing words, write out the word first
and then say it out loud
 
• Write out choices for the person with PPA and let them choose the appropriate
response
 
• Write out conversational scripts to use during specific situations (e.g. ordering a
favorite drink at a coffee shop)
 
Gestures
• Gestures can be developed for core daily functions
 
• Practice and have gestures in place, before they are actually needed
Body Language
 
• So much of communication is non-verbal!
 
• Use facial expression and the sense of touch when communication with
someone with PPA
 
• Give a firm handshake, a pat on the back, hold hands, hug, sit close
 
Communication is a two-way street. Both diagnosed persons and their communication
partners will need to adapt to new methods of communication. Working with a speechlanguage
pathologist can help make this transition. Remember, the goal is
communication, not perfection.
 
Family & Friends
Caring for Someone with PPA: Reaching Out and Getting the Help You Need
While Alzheimer’s disease strikes a majority of people in their later years, primarily age
65 and over, this fact tends to overshadow those who become affected by dementia at
an earlier age. Primary Progressive Aphasia (PPA) typically strikes people in the 50’s
and 60’s. The early age of onset presents patients and families with unique issues and
stresses.
 
Due to the rarity of PPA, you may have had difficulty obtaining an accurate diagnosis.
Once that diagnosis is obtained, there is the task of finding as much information as
possible about PPA. At present, there are few resources available. Much of the material
provided to caregivers, families and friends of patients is directed toward an older
population. Additionally, the programs and services have been geared to meet the needs
of that population.
 
How will PPA affect my friend or family member’s job?
Employment is often the first issue that must be addressed with someone who has been
diagnosed with PPA. A professional person in their 50’s and 60’s is typically at the peak
of their career or just approaching retirement, perhaps not ready financially or prepared
emotionally to stop working. They may not have finished building their “nest egg” for
retirement and may have expenses—such as their children’s education—which rely on
those additional years of employment.
 
Since language and verbal skills are essential for most jobs, the need to terminate
employment is inevitable and planning for that is necessary.
 
Immediate application for social security disability should be completed, although
navigating one’s way through this bureaucratic system can be daunting. The Alzheimer’s
Association has a Disability Documentation Kit that may make your passage a little
easier. They can be reached at 1-800-272-3900. If the application for social security is
denied, work with your physician to clearly document the diagnosis and advocate on
your behalf by appealing the decision. After receiving social security disability benefits
for two years, persons under 65 years of age are eligible for Medicare.
 
What happens after my family member or friend with PPA is no longer employed?
One of the most common difficulties that arises following termination of employment is
finding something valuable to do with the time once spent working. The well spouse may
need to continue or return to working outside the home, perhaps seek employment for
the first time. The person diagnosed with PPA is experiencing a tumultuous role change
from that of an independent, self-sufficient person to that of someone who is becoming
increasingly dependent on others. Adult day services programs that have proliferated in
number and may provide dementia specific care, tend to focus their efforts on older
adults with memory loss. Often, a PPA patient and family feel uncomfortable with this
type of a setting. However, persons with PPA have much to contribute and with proper
guidance and supervision can remain active in a variety of areas. This takes creativity
and perseverance on the part of families. Contacting your local Department of
Rehabilitation Services/Department of Human Services is the first step. Call 1-800-275-
3677 for the office nearest you.
 
What about the family?
There are also unique family issues due to the younger age at which PPA typically
occurs. Developmentally, PPA occurs at an age where a chronic illness of this
magnitude is unexpected. The well spouse may have the stress of being pulled between
work and family, may also be at the peak of their career and/or have young or
adolescent children requiring their attention and care. Additionally, these children are
experiencing the loss of their parent as they once knew him or her and will need ongoing
emotional support and attention as they grieve this loss. Watch for signs of extreme
behaviors: withdrawal, apathy, poor school performance, or increased aggression. It
may be helpful to seek a counselor experienced in family therapy to give children a safe
place to express their feelings and help the well spouse in their new role of single parent.
Recognize, too, that the loss of one’s relationship with a spouse to this disease over a
gradual period of time can raise many different emotions: confusion, anger, guilt, and
sadness, to name a few. The role of partner becomes that of caregiver. It is important to
remain in touch with the emotions that can arise and seek counseling for support and
assistance in coping as needed.
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