Introduction to frontotemporal dementia

The symptoms of frontotemporal dementia (FTD) and other frontotemporal disorders gradually rob people of basic abilities — thinking, talking, walking, and socializing — that most of us take for granted. FTD and other frontotemporal disorders are a common cause of early-onset dementia. 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.

There is currently no cure for FTD or related disorders, and no treatments to slow or stop the progression of the disease, but there are ways to help manage the symptoms. Research is improving our awareness and understanding of these challenging conditions. This progress is opening doors to better diagnosis, improved care, and eventually, new treatments.

Frontotemporal disorders are the result of damage to neurons primarily in the in the frontal and temporal lobes of the brain. As neurons die in these regions, the frontal and temporal 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 movement and walking. FTD and related disorders are rare and tend to occur at a younger age than other forms of dementia. Roughly 60% of people with FTD are 45 to 64 years old.

FTD is progressive, meaning symptoms get worse over time. In the early stages, people may have just one type of symptom. As the disease progresses, other symptoms will appear as more parts of the brain are affected. It is difficult to predict how long someone with FTD will live. Some people live more than 10 years after diagnosis, while others live less than two years after they are diagnosed.

FTD-related changes in the brain

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, 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 help people avoid inappropriate social behaviors, such as shouting loudly in a library or at a funeral. They aid in making 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. 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 and 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 aren’t working properly, people may have difficulty recognizing and responding appropriately to emotions.

Which lobe — and which part of the lobe — is affected first determines which symptoms appear initially. For example, if the disease starts in the part of the frontal lobe responsible for decision-making, then the first symptom might be trouble 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.

In rare cases, frontotemporal disorders can begin in a different part of the brain. For example, when patients have early movement problems, such as falls, swallowing difficulties or muscle stiffness, the disease may be beginning in the movement parts of the brain such as the basal ganglia or brainstem.

Frontotemporal disorders (FTD), sometimes called frontotemporal dementia, are the result of damage to neurons in the frontal and temporal lobes of the brain. Many possible symptoms can result, including unusual behaviors, emotional problems, trouble communicating, difficulty with work, or difficulty with walking. FTD is rare and tends to occur at a younger age than other forms of dementia. Roughly 60% of people with FTD are 45 to 64 years old.

FTD is progressive, meaning symptoms get worse over time. In the early stages, people may have just one symptom. As the disease progresses, other symptoms appear as more parts of the brain are affected. It is difficult to predict how long someone with FTD will live. Some people live more than 10 years after diagnosis, while others live less than two years after they are diagnosed.

There is currently no cure for FTD, and no treatments slow or stop the progression of the disease, but there are ways to help manage the symptoms.

What do the terms mean?

One of the challenges shared by people living with these disorders, families, clinicians, and researchers is what terminology to use. Here, we have used the term frontotemporal disorders to characterize this group of diseases and the abbreviation FTD, which is commonly used to refer to them. Other terms used include frontotemporal lobar degeneration and frontotemporal dementia, but it's important to note that with some frontotemporal disorders, the primary symptoms are problems with speech or movement, rather than dementia symptoms. Physicians and psychologists diagnose the different forms of FTD based on a person’s symptoms as well as the results of brain scans and genetic tests.

What are the types and symptoms of FTD?

In the early stages, it can be hard to know which type of FTD a person has because symptoms and the order in which they appear can vary from one person to another. Also, the same symptoms can appear across different disorders and vary from one stage of the disease to the next as different parts of the brain are affected.

Symptoms of FTD are often misunderstood. Family members and friends may think that a person is misbehaving, leading to anger and conflict. It is important to understand that people with these disorders cannot control their behaviors and other symptoms and lack any awareness of their illness.

There are three types of frontotemporal disorders (FTD): behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and movement disorders.

Behavioral variant frontotemporal dementia

The most common FTD, bvFTD, involves changes in personality, behavior, and judgment. People with this disorder may have problems with cognition, but their memory may stay relatively intact. Symptoms can include:

• Problems planning and sequencing (thinking through which steps come first, second, and so on)
• Difficulty prioritizing tasks or activities
• Repeating the same activity or saying the same word over and over
• Acting impulsively or saying or doing inappropriate things without considering how others perceive the behavior
• Becoming disinterested in family or activities they used to care about

Over time, language and/or movement problems may occur, and the person living with bvFTD will need more care and supervision.

Primary progressive aphasia

PPA involves changes in the ability to communicate — to use language to speak, read, write, and understand what others are saying. This includes difficulty using or understanding words (aphasia) and difficulty speaking properly (e.g., slurred speech). People with PPA may have one or both of these symptoms. They may become mute or unable to speak.

Many people with PPA develop symptoms of dementia. 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 that appear first.

• Semantic PPA: A person slowly loses the ability to understand single words and sometimes to recognize the faces of familiar people and common objects.
• Agrammatic PPA: A person has more and more trouble speaking and may omit words that link nouns and verbs (such as to, from, the). Eventually, the person may no longer be able to speak at all. The person may eventually develop movement symptoms similar to those seen in corticobasal syndrome.
• Logopenic PPA: A person has trouble finding the right words during a conversation but can understand words and sentences. The person does not have problems with grammar.

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 changes in the brain that cause them.

Movement disorders

Two rare neurological movement disorders associated with FTD, corticobasal syndrome and progressive supranuclear palsy, occur when the parts of the brain that control movement are affected. The disorders may affect thinking and language abilities, too.

• Corticobasal syndrome can be caused by corticobasal degeneration — a gradual atrophy (shrinkage) 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 apraxia, the inability to use the hands or arms to perform a movement despite normal strength, such as difficulty closing buttons or operating small appliances. Other symptoms can include muscle rigidity and difficulty swallowing. Symptoms may appear first on one side of the body, but eventually both sides are affected. Occasionally, a person with corticobasal syndrome first has language problems or trouble orienting objects in space and later develops movement symptoms. Not everyone who has corticobasal syndrome has problems with memory, cognition, language, or behavior.
• Progressive supranuclear palsy 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 this disorder is trouble with eye movements, particularly looking down. These symptoms may give the face a fixed stare. Problems with behavior, memory, problem solving, and judgment can also develop.

Other movement-related types of FTD 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 genetic variant. 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, also called FTD with motor neuron disease, is a combination of bvFTD and ALS, the latter commonly known as Lou Gehrig’s disease. In addition to the behavioral and/or language changes seen in bvFTD, people with FTD-ALS experience the progressive muscle weakness seen in ALS, fine jerks, and wiggling in muscles. Symptoms of either disease may appear first, with other symptoms developing over time. Changes in certain genes have been found in some people with FTD-ALS, though most cases are not hereditary.

What causes FTD?

Scientists are beginning to understand the biological and genetic basis for the changes observed in brain cells that lead to FTD.

Scientists describe FTD using the 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 FTD is unknown. Individuals with a family history of FTD are more likely to develop such a disorder. About 10 to 30% of bvFTD is due to specific genetic causes.

FTD that runs in a family is often related to variants (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 FTD:

• Tau gene (also called the MAPT gene) — A change in this gene causes abnormalities in a protein called tau, which then forms tangles inside neurons and ultimately leads to the destruction of brain cells. Inheriting a variant 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.
• GRN gene — A change in this gene can lead to lower production of the protein progranulin, which in turn causes another protein, TDP-43, to go awry in brain cells. Many frontotemporal disorders can result, though bvFTD is the most common. The GRN gene can cause different symptoms in different family members and cause the disease to begin at different ages.
• C9ORF72 gene — An unusual change in this gene appears to be the most common genetic abnormality in familial frontotemporal disorders and familial ALS. This variant can cause a frontotemporal disorder, ALS, or both conditions.

In recent years researchers have discovered several other genetic changes in genes that lead to rare familial types of frontotemporal disorders. These other variants account for less than 5% of all cases of FTD.

Families affected by inherited and familial forms of FTD can help scientists advance research by participating in clinical studies and trials.

How is FTD diagnosed?

FTD can be hard to diagnose because the symptoms are similar to those of other conditions. For example, bvFTD is sometimes misdiagnosed as a mood disorder, such as depression. To make matters more confusing, a person can have both FTD and another type of dementia, such as Alzheimer's disease. Also, because these disorders are rare, physicians may be unfamiliar with the signs and symptoms.

To help diagnose frontotemporal dementia, a doctor may:

• Perform an exam and ask about symptoms
• Look at personal and family medical history
• Use laboratory tests to help rule out other conditions
• Order genetic testing
• Conduct tests to assess memory, thinking, language skills, and physical functioning
• Order imaging of the brain

A psychiatric evaluation can help determine if depression or another mental health condition is causing or contributing to the condition. Only genetic tests in familial cases or a brain autopsy after a person dies can confirm a diagnosis of FTD.

Researchers are studying ways to diagnose FTD earlier and more accurately and to distinguish them from other types of dementia. One area of research involves biomarkers, such as proteins or other substances in the blood or cerebrospinal fluid which can be used to measure disease progression or the effects of treatment. Researchers are also exploring ways to improve brain imaging and neuropsychological testing.

Treatment and management of FTD

So far, there is no cure for FTD and no way to slow down or prevent these diseases. 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 changes in FTD

Behavior changes associated with bvFTD can upset and frustrate family members and other caregivers. Understanding changes in personality and behavior and knowing how to respond can reduce frustration and help provide the best care for a person with FTD.

Managing behavioral symptoms can involve several approaches. Here are some strategies to consider:

• Try to accept rather than challenge someone with behavioral symptoms. Arguing or reasoning will not help, because they cannot control their behaviors or see that they are unusual or upsetting to others. Instead, be as sensitive as possible and understand that it’s the illness “talking.”
• Take a “timeout” when frustrated — 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, such as “What do you want to do today?” are more difficult to answer than specific ones, such as “Do you want to go to the park or for a walk?”.
• Maintain a regular schedule, reduce distractions, and modify the environment to reduce confusion and improve the person’s sleep.
• If compulsive eating is an issue, consider supervising eating, limiting food choices, locking cabinets and the refrigerator, and distracting the person with other activities.

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.

Medications are available to treat certain behavioral symptoms. Antidepressants called selective serotonin reuptake inhibitors are commonly prescribed to treat social disinhibition and impulsive behavior. People with aggression or delusions sometimes take low doses of antipsychotic medications. If a particular medication is not working, a doctor may try another. Always consult a doctor before changing, adding, or stopping a drug or supplement.

Treating language problems in FTD

Treatment of 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. The following strategies may help:

• Use a communication notebook (an album of photos labeled with names of people and objects), gestures, and drawings to communicate without talking.
• Store lists of words or phrases in a computer or phone to point to.
• Speak slowly and clearly, use simple sentences, wait for responses, and ask for clarification if needed.
• Work with a speech-language pathologist familiar with PPA to determine the best tools and strategies to use. Note that many speech-language pathologists are trained to treat aphasia caused by stroke, which requires different strategies from those used with PPA.

Managing movement problems in FTD

Medications and physical and occupational therapy may provide modest relief for the movement symptoms of FTD. A doctor who specializes in these disorders can guide treatment.

For people with corticobasal syndrome, Parkinson’s disease medicines may offer some temporary improvement. Physical and occupational therapy may help the person move more easily. Speech therapy can help them manage language symptoms.

For people with progressive supranuclear palsy, 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 two to three 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.

Physicians, nurses, social workers, and physical, occupational, and speech therapists who are familiar with these conditions 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 FTD treatment

Researchers are continuing to explore the biological changes in the body, including genetic variants and proteins, that lead to FTD and identify and test possible new drugs and other treatments. They are also developing better ways to track disease progression, so that treatments, when they become available, can be directed to the right people. Clinical trials and studies are underway to advance these efforts. People with FTD and healthy people may be able to participate.

Dementia is not a specific disease but is rather a general term for the impaired ability to remember, think, or make decisions that interferes with doing everyday activities. Alzheimer’s disease is the most common type of dementia. Though dementia mostly affects older adults, it is not a part of normal aging.

How common is dementia?

Of those at least 65 years of age, there is an estimated 5.0 million adults with dementia in 2014 and projected to be nearly 14 million by 2060.

Isn’t dementia part of normal aging?

No, many older adults live their entire lives without developing dementia. Normal aging may include weakening muscles and bones, stiffening of arteries and vessels, and some age-related memory changes that may show as:

• Occasionally misplacing car keys
• Struggling to find a word but remembering it later
• Forgetting the name of an acquaintance
• Forgetting the most recent events

Normally, knowledge and experiences built over years, old memories, and language would stay intact.

What are the signs and symptoms of dementia?

Because dementia is a general term, its symptoms can vary widely from person to person. People with dementia have problems with:

• Memory
• Attention
• Communication
• Reasoning, judgment, and problem solving
• Visual perception beyond typical age-related changes in vision

Signs that may point to dementia include:

• Getting lost in a familiar neighborhood
• Using unusual words to refer to familiar objects
• Forgetting the name of a close family member or friend
• Forgetting old memories
• Not being able to complete tasks independently

What increases the risk for dementia?

• Age
  The strongest known risk factor for dementia is increasing age, with most cases affecting those of 65 years and older

• Family history
  Those who have parents or siblings with dementia are more likely to develop dementia themselves.

• Race/ethnicity
  Older African Americans are twice more likely to have dementia than whites. Hispanics 1.5 times more likely to have dementia than whites.

• Poor heart health
  High blood pressure, high cholesterol, and smoking increase the risk of dementia if not treated properly.

• Traumatic brain injury
  Head injuries can increase the risk of dementia, especially if they are severe or occur repeatedly.

How is dementia diagnosed?

A healthcare provider can perform tests on attention, memory, problem solving and other cognitive abilities to see if there is cause for concern. A physical exam, blood tests, and brain scans like a CT or MRI can help determine an underlying cause.

What are the most common types of dementia?

• Alzheimer’s disease. This is the most common cause of dementia, accounting for 60 to 80 percent of cases. It is caused by specific changes in the brain. The trademark symptom is trouble remembering recent events, such as a conversation that occurred minutes or hours ago, while difficulty remembering more distant memories occurs later in the disease. Other concerns like difficulty with walking or talking or personality changes also come later. Family history is the most important risk factor. Having a first-degree relative with Alzheimer’s disease increases the risk of developing it by 10 to 30 percent.

• Vascular dementia. About 10 percent of dementia cases are linked to strokes or other issues with blood flow to the brain. Diabetes, high blood pressure and high cholesterol are also risk factors. Symptoms vary depending on the area and size of the brain impacted. The disease progresses in a step-wise fashion, meaning symptoms will suddenly get worse as the individual gets more strokes or mini-strokes.

• Lewy body dementia. In addition to more typical symptoms like memory loss, people with this form of dementia may have movement or balance problems like stiffness or trembling. Many people also experience changes in alertness including daytime sleepiness, confusion or staring spells. They may also have trouble sleeping at night or may experience visual hallucinations (seeing people, objects or shapes that are not actually there).

• Fronto-temporal dementia. This type of dementia most often leads to changes in personality and behavior because of the part of the brain it affects. People with this condition may embarrass themselves or behave inappropriately. For instance, a previously cautious person may make offensive comments and neglect responsibilities at home or work. There may also be problems with language skills like speaking or understanding.

• Mixed dementia. Sometimes more than one type of dementia is present in the brain at the same time, especially in people aged 80 and older. For example, a person may have Alzheimer’s disease and vascular dementia. It is not always obvious that a person has mixed dementia since the symptoms of one type of dementia may be most prominent or may overlap with symptoms of another type. Disease progression may be faster than with one kind of dementia.

• Reversible causes. People who have dementia may have a reversible underlying cause such as side effect of medication, increased pressure in the brain, vitamin deficiency, and thyroid hormone imbalance. Medical providers should screen for reversible causes in patients who are concerning for dementia.

How is dementia treated?

Treatment of dementia depends on the underlying cause. Neurodegenerative dementias, like Alzheimer’s disease, have no cure, though there are medications that can help protect the brain or manage symptoms such as anxiety or behavior changes. Research to develop more treatment options is ongoing.

Leading a healthy lifestyle, including regular exercise, healthy eating, and maintaining social contacts, decreases chances of developing chronic diseases and may reduce number of people with dementia.

What to do if a loved one is suspicious of having dementia?

• Discuss with loved one. Talk about seeing a medical provider about the observed changes soon. Talk about the issue of driving and always carrying an ID.
• Medical assessment. Be with a provider that you are comfortable with. Ask about the Medicare Annual Wellness exam.
• Family Meeting. Start planning, and gather documents like the Health Care Directive, Durable Power of Attorney for Health Care, Estate Plan.

Types and symptoms of frontotemporal disorders

Frontotemporal disorders can be grouped into three types, defined by the earliest symptoms. In the early stages, it can be hard to know which type of FTD a person has because symptoms and the order in which they appear can vary from one person to another. Also, the same symptoms can appear across different disorders and vary from one stage of the disease to the next as different parts of the brain are affected. In general, changes in the frontal lobe are associated with behavioral symptoms, while changes in the temporal lobe affect language and emotions. Because it is rare and not well understood, FTD is sometimes misdiagnosed as Alzheimer’s disease or other types of dementia.

Symptoms of FTD are often misunderstood. Family members and friends may think that a person is misbehaving, leading to anger and conflict. It is important to understand that people with these disorders cannot control their behaviors and other symptoms and (before they are diagnosed) lack any awareness of their illness.

Behavioral variant frontotemporal dementia

The most common FTD, behavioral variant frontotemporal dementia (bvFTD), involves changes in personality, behavior, and judgment. People with bvFTD 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. People with this disorder may or may not have problems with cognition or memory. Symptoms can include:

• Problems planning and sequencing
• Difficulty prioritizing tasks or activities
• Repeating the same activity or saying the same word over and over
• Acting impulsively or saying or doing inappropriate things without considering how others perceive the behavior
• Becoming uninterested in family or activities they used to care about
• Displaying flat, exaggerated, or improper emotions that seem disconnected from the situation
• Difficulty reading social signals, seeming to lack empathy
• Compulsive eating or taking food from others’ plates

Over time, language and/or movement problems may occur, and the person living with bvFTD will need more care and supervision.

Primary progressive aphasia

Primary progressive aphasia (PPA) involves changes in the ability to communicate — to use language to speak, read, write, and understand what others are saying. This includes difficulty using or understanding words (aphasia) and difficulty speaking properly (e.g., slurred speech). People with PPA may have one or both of these symptoms. They may eventually become unable to speak.

Many people with PPA develop symptoms of dementia. Problems with memory, reasoning, and judgment are not apparent at first but can develop over time. 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 language problems that appear first.

• Semantic PPA: A person slowly loses the ability to understand single words and sometimes to recognize the faces of familiar people and common objects.

• Agrammatic PPA: A person has more and more trouble speaking and may omit words that link nouns and verbs, such as to, from, and the. Eventually, the person may no longer be able to speak at all. The person may later develop movement symptoms similar to those seen in corticobasal syndrome (see below).
• Logopenic PPA: A person has trouble finding the right words during a conversation but can understand words and sentences. The person does not have problems with grammar. Logopenic PPA is usually accompanied by the hallmark brain changes seen in Alzheimer’s disease.

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 changes in the brain that cause them.

Movement disorders

Two rare neurological movement disorders associated with FTD, corticobasal syndrome and progressive supranuclear palsy, occur when the parts of the brain that control movement are damaged. The disorders may affect thinking and language abilities, too.

• Corticobasal syndrome can be caused by corticobasal degeneration — a gradual atrophy (shrinking) and loss of nerve cells in specific parts of the brain. This causes progressive loss of the ability to control movement, typically beginning around age 60. The most prominent symptom may be apraxia, the inability to use the hands or arms to perform a movement despite normal strength, such as difficulty fastening buttons or operating small appliances. Other symptoms can include muscle rigidity and difficulty swallowing. Symptoms may appear first on one side of the body, but eventually both sides are affected. Occasionally, a person with corticobasal syndrome first has language problems or trouble orienting objects in space and later develops movement symptoms. Not everyone who has corticobasal syndrome has problems with memory, cognition, language, or behavior.
• Progressive supranuclear palsy typically 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 this disorder is trouble with eye movements, particularly looking down. These symptoms may give the face a fixed stare. Problems with behavior, language, problem solving, and judgment can also develop.

Other movement-related types of FTD include frontotemporal dementia with parkinsonism and frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS).

• Frontotemporal dementia with parkinsonism is usually caused by CBS or PSP, but rarely can be an inherited disease caused by a genetic variant in the tau protein also called the MAPT gene. Symptoms are similar to those of Parkinson’s disease and include slowed movement, stiffness, and balance problems, and changes in behavior or language.
• FTD-ALS, also called FTD with motor neuron disease, is a combination of bvFTD and ALS, with the latter sometimes known as Lou Gehrig’s disease. In addition to the behavioral and/or language changes seen in bvFTD, people with FTD-ALS experience the progressive muscle weakness seen in ALS, fine jerks, and wiggling in muscles (fasciculation). Symptoms of either disease may appear first, with other symptoms developing over time. People with FTD-ALS experience the progressive muscle weakness seen in ALS, such as fine jerks and wiggling in muscles. Symptoms of either disease may appear first, with other symptoms developing over time. Changes in certain genes have been found in some people with FTD-ALS.

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 for 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.
• Compulsive eating—Gorging on food, especially starchy foods like bread and cookies, or taking food from other people's plates.
• 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.

Scientists are beginning to understand the biological and genetic basis for the changes observed in brain cells that lead to FTD

.

Scientists describe FTD using the 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 FTD is unknown. Individuals with a family history of FTD are more likely to develop such a disorder. About 10 to 30% of bvFTD is due to specific genetic causes.

FTD that runs in a family is often related to variants (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 FTD:

• Tau gene (also called the MAPT gene) — A change in this gene causes abnormalities in a protein called tau, which then forms tangles inside neurons and ultimately leads to the destruction of brain cells. Inheriting a variant 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.
• GRN gene — A change in this gene can lead to lower production of the protein progranulin, which in turn causes another protein, TDP-43, to go awry in brain cells. Many frontotemporal disorders can result, though bvFTD is the most common. The GRN gene can cause different symptoms in different family members and cause the disease to begin at different ages.
• C9ORF72 gene — An unusual change in this gene appears to be the most common genetic abnormality in familial frontotemporal disorders and familial ALS. This variant can cause a frontotemporal disorder, ALS, or both conditions.

In recent years researchers have discovered several other genetic changes in genes that lead to rare familial types of frontotemporal disorders. These other variants account for less than 5% of all cases of FTD.

Families affected by inherited and familial forms of FTD can help scientists advance research by participating in clinical studies and trials.

Brian's story

Brian, an attorney, began having trouble organizing his cases. In time, his law firm assigned him to do paperwork only. Brian’s wife thought he was depressed because his father had died two years earlier. Brian, 56, was treated for depression, but his symptoms got worse. He became more disorganized and began making sexual comments to his wife’s female friends. Even more unsettling, he neither understood nor cared that his behavior disturbed his family and friends. As time went on, Brian had trouble paying bills and was less affectionate toward his wife and young son. Three years after Brian’s symptoms began, his counselor recommended a neurological evaluation. Brian was diagnosed with behavioral variant FTD — the most common form of FTD.

The underlying causes of frontotemporal dementia are not yet fully understood. People with FTD have abnormal amounts or forms of proteins called tau and TDP-43 inside nerve cells, or neurons, in their brain. The neurons die because the buildup of these proteins causes damage. In some cases, changes or differences in genes can be identified as the cause of FTD. For example, about 10% to 30% of bvFTD cases can be attributed to genetic causes. People with a family history of FTD are more likely to have a genetic form of the disease.

How is FTD diagnosed?

FTD can be hard to diagnose because the symptoms resemble those of other conditions. For example, bvFTD is sometimes misdiagnosed as a mood disorder, such as depression. To make matters more confusing, a person can have both FTD and another type of dementia, such as Alzheimer's disease. Also, because these disorders are rare, clinicians may be unfamiliar with the signs and symptoms.

To help diagnose FTD, a doctor may:

• Perform an exam and ask about symptoms
• Review personal and family medical history
• Use laboratory tests to help rule out other conditions, including Alzheimer’s disease
• Order genetic testing
• Conduct evaluations to assess behavior, memory, thinking, language skills, and physical function
• Order imaging of the brain

A psychiatric evaluation can help determine if depression or another mental health condition is causing or contributing to the condition. A diagnosis of FTD can only be confirmed by genetic tests (in familial cases) or a brain autopsy after death.

Getting the wrong diagnosis can be frustrating. Without an accurate diagnosis, people with frontotemporal dementia and related disorders may not get appropriate treatment to manage their symptoms. Families may not get the help they need. The medical centers listed at the end of this webpage can help people with FTD get diagnosis and treatment.

Researchers are studying ways to diagnose FTD earlier and more accurately and to distinguish them from other types of dementia. One area of research involves biomarkers, such as proteins or other substances in the blood or cerebrospinal fluid, which can be used to help diagnose FTD and measure disease progression. Results from biomarker tests also can help rule out other causes of dementia. For example, a blood test for Alzheimer’s disease could help establish whether the cause of dementia is likely to be Alzheimer’s or something else. Researchers are also exploring ways to improve brain imaging and neuropsychological testing.

How is FTD treated and managed?

So far, there is no cure for FTD and no way to slow down or prevent it. 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 changes in FTD

Behavior changes associated with bvFTD can upset and frustrate family members and other caregivers. Understanding changes in personality and behavior and knowing how to respond can reduce frustration and help provide the best care for a person with FTD. Finding expert assistance can be helpful in learning the best strategies to manage behavior changes. See the section at the end of this page for information on resources that can connect patients and families with experts to provide support.

Managing behavioral symptoms can involve several approaches. Here are some strategies to consider:

• Try to accept rather than challenge someone with behavioral symptoms. Arguing or reasoning will not help, because they cannot control their behaviors or see that they are unusual or upsetting to others. Instead, be as sensitive as possible and understand that it’s the illness “talking.”
• Take a “timeout” when frustrated — take deep breaths, count to 10, or leave the room for a few minutes.
• To deal with apathy, limit choices and offer specific options. Open-ended questions, such as “What do you want to do today?” are more difficult to answer than specific ones, such as “Do you want to go to the park or for a walk?”.
• Maintain a regular schedule, reduce distractions, and modify the environment to reduce confusion and improve the person’s sleep.
• If compulsive eating is an issue, consider supervising eating, limiting food choices, locking cabinets and the refrigerator, and distracting the person with other activities.

To ensure the safety of the person and his or her family, caregivers may have to take on new responsibilities or arrange care that was not needed before.

Medications are available to treat certain behavioral symptoms. Antidepressants called selective serotonin reuptake inhibitors are commonly prescribed to treat social disinhibition and impulsive behavior. People with aggression or delusions sometimes take low doses of antipsychotic medications. If a particular medication is not working, a doctor may try another. Always consult a doctor before changing, adding, or stopping a drug or supplement.

Treating language problems in Primary Progressive Aphasia and FTD

Treatment of 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 in people with PPA, different strategies may be needed as the illness progresses. The following may help:

• Use a communication notebook (an album of photos labeled with names of people and objects), gestures, and drawings to communicate without talking.
• Store lists of words or phrases in a computer or smartphone to point to.
• Speak slowly and clearly, use simple sentences, wait for responses, and ask for clarification if needed.
• Work with a speech-language pathologist familiar with PPA to determine the best tools and strategies to use. Note that many speech-language pathologists are trained to treat aphasia caused by stroke, which requires different strategies from those used with PPA.

Mary Ann's story

Mary Ann, a television news anchor for 20 years, began having trouble reading the nightly news. At first, her doctor thought she had a vision problem, but tests showed that her eyesight was normal. Although normally creative and energetic, Mary Ann, 52, had trouble finishing assignments and voicing her ideas at staff meetings. In time, she was let go from her job. Mary Ann applied for Social Security disability benefits, which required a medical exam. Her symptoms puzzled several doctors until a neurologist diagnosed logopenic PPA. A speech therapist taught Mary Ann to use a personal digital assistant to express words and phrases. For emergencies, Mary Ann carries a card in her wallet that explains her condition.

Managing movement problems in FTD

Medications and physical and occupational therapy may provide modest relief for the movement symptoms of FTD and related frontotemporal disorders. A doctor who specializes in FTD can guide treatment.

For people with PSP, Parkinson’s disease medicines may offer some temporary improvement. Physical and occupational therapy may help the person move more easily. Speech therapy can help them manage language symptoms.

For people with progressive supranuclear palsy, 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 rolling walker with brakes — can help maintain balance. Speech, vision, and swallowing difficulties usually do not respond to any drug treatment; however antidepressants have shown modest success, and patients with uncontrollable laughing or crying often respond to medications for these problems. For people with abnormal eye movements, special glasses called prisms are sometimes prescribed.

People with FTD-ALS typically decline quickly over two to three 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.

Physicians, nurses, social workers, and physical, occupational, and speech therapists who are familiar with these conditions can ensure that people with movement disorders get appropriate medical treatment and that their caregivers can help them live as well as possible.

What is the future of FTD treatment?

Researchers are continuing to explore the biological changes in the body, including genetic variants and proteins, that lead to FTD and identify and test possible new drugs and other treatments. They also want to develop better ways, such as specialized brain imaging, to track progression, so that treatments, when they become available, can be directed to the people for whom they will work best.

Clinical trials and studies are underway to advance these efforts. People with FTD and healthy people may be able to participate. The FTD Disorders Registry is a contact and research registry for people diagnosed with frontotemporal disorders. It is open to family members, caregivers, or friends of people diagnosed with FTD. Registries help speed up trials by gathering a list of “willing and able” potential participants. People who sign up may be referred to specific studies but are not obligated to participate.

Strategies to help manage FTD symptoms

There is no cure for FTD and no way to slow it down or prevent it. However, there are ways to help manage symptoms.

• Managing behavior changes in FTD. Try to recognize it’s the illness “talking” and accept rather than challenge people with behavioral symptoms. Arguing or reasoning with the person will not help; they cannot control their behaviors or even see that they are unusual or upsetting to others.
• Treating language problems in FTD. To help with language issues, speak slowly and clearly, use simple sentences, wait for responses, and ask for clarification if you don’t understand something. Gesturing, drawing, and using an album with labeled photos of people and objects may help communicate without talking. A speech-language pathologist can help determine the best tools and strategies for a particular person.
• Managing movement problems in FTD. Several types of FTD cause problems with movement, including difficulty balancing, walking, and swallowing. Medications and physical and occupational therapy may provide modest relief for the movement symptoms of FTD. A doctor who specializes in these disorders can guide treatment.

How FTD and related disorders affect families

People with FTD and their families must cope with changing relationships, especially as symptoms get worse. Spouses or partners may find themselves not only taking on caregiving responsibilities, but also household responsibilities that their partner can no longer perform. Children may suffer the gradual loss of a parent before they reach adulthood. Family members and friends may feel alienated or embarrassed by the person's behavior. Life at home can become very stressful.

How FTD can affect work

People living with FTD may have difficulty with basic work skills, such as organizing, planning, and following through on tasks. Activities that were easy before might take much longer or become impossible. People may 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 their hours, or even quit working 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 FTD can qualify for Social Security disability benefits through the “Compassionate Allowances” program (800-772-1213), a program that helps individuals with certain serious conditions access benefits quickly.

FTD caregiver health and support

Caring for someone with FTD presents unique challenges. Many caregivers face declines in their own health while caring for a person with FTD or a related disorder. To stay healthy, caregivers can:

• Get regular healthcare.
• 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 daycare 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 can be a valuable resource to share experiences and tips with others who may be in a similar situation. Nonprofit organizations and community organizations may provide online or in-person support groups.

The organizations listed at the end of this webpage can help with information about caregiver services and support.

Long-term care for people with FTD

For many caregivers, there comes a point when they can no longer take care of the person with FTD without professional help. The caregiving demands are simply too great, and the person may need around-the-clock care. As the disease progresses, caregivers may want to get home health care 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 FTD 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 their loved one.

End-of-life concerns

People with FTD typically live six to eight 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 FTD or a related disorder. Early on, many people can understand and participate in legal decisions. But as their illness progresses, it becomes harder to make such decisions.

A doctor 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.

It is impossible to predict the exact course of frontotemporal dementia and other 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. You can help pave the way for new and more effective treatments for people living with FTD by joining a clinical trial.