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What Are The Symptoms Of A Frontotemporal Dementia

Who Gets Frontotemporal Dementia

Frontotemporal Dementia, Causes, Signs and Symptoms, Diagnosis and Treatment.

FTD can affect anybody. It typically affects people at a younger age than Alzheimers disease, with symptoms beginning in the 50s or 60s, and sometimes younger.

Almost a third of people with FTD have a family history of dementia. However, only about 10-15% of cases have familial FTD, in which a gene mutation is passed on that causes the disease.

The genetic basis of FTD is not fully understood and is actively being researched.

Language Variant Of Frontotemporal Dementia

Two other forms of FTD are described: non-fluent aphasia features a progressive difficulty producing the lip and tongue movements required in order to speak. Speech becomes effortful, slow, and ungrammatical. Instead of producing normal complicated sentences, the person with non-fluent aphasia often speaks in short phrases, missing the connecting prepositions and including wrong words. Understanding of single words is preserved, but long sentences are likely to be overwhelming. Eventually, language expression may be completely lost, resulting in muteness. In the logopenic variant, people have trouble finding words and speak more hesitantly as they search for the correct word. They are able to understand the meaning of words and their speech is not labored. Repetition is difficult for them and they sometimes substitute meaningless sounds for words.

What Are The Complications Of Frontotemporal Dementia

FTD is not life-threatening people may live with it for years. But it can lead to an increased risk for other illnesses that can be more serious. Pneumonia is the most common cause of death, with FTD. People are also at increased risk for infections and fall-related injuries.

As FTD progressively worsens, people may engage in dangerous behaviors or be unable to care for themselves. They may need 24-hour nursing care or to stay in an assisted living facility or nursing home.

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Behaviour And Personality Changes

Many people with frontotemporal dementia develop a number of unusual behaviours they’re not aware of.

These can include:

  • losing interest in people and things
  • losing drive and motivation
  • inability to empathise with others, seeming cold and selfish
  • repetitive behaviours, such as humming, hand-rubbing and foot-tapping, or routines such as walking exactly the same route repetitively
  • a change in food preferences, such as suddenly liking sweet foods, and poor table manners
  • compulsive eating, alcohol drinking and/or smoking
  • neglecting personal hygiene

As the condition progresses, people with frontotemporal dementia may become socially isolated and withdrawn.

What Are The Stages Of Frontotemporal Dementia

Frontotemporal Dementia

James M. Ellison, MD, MPH

Swank Center for Memory Care and Geriatric Consultation, ChristianaCare

  • Expert Advice

Learn about the symptoms associated with the early, middle, and late stages of frontotemporal dementia.

On the surface, Joseph, Barbara, and Lloyd may not seem to be experiencing related changes. Each of them, though, turned out to be in the early stage of frontotemporal dementia . FTD is the diagnosis for about 5 percent of people with major neurocognitive disorders . About 70 percent of cases begin before age 65, so it is a more common dementia among the young old. FTD involves degeneration of the frontal and temporal lobes of the brain. The frontal lobes are important regulators of behavior and the temporal lobes assist in our understanding and expression of language. The symptoms of FTD, therefore, include major changes in behavior, impairment of language, or both.

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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
  • 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.

How Is Frontotemporal Dementia Treated

Currently, no treatments are available to cure or slow the progression of FTD, but healthcare providers may prescribe medicine to treat symptoms. Antidepressants may help treat anxiety and control obsessive-compulsive behaviors and other symptoms. Prescription sleeping aids can help ease insomnia and other sleep disturbances. Antipsychotic medicine may reduce irrational and compulsive behaviors.

Behavior modification may help control unacceptable or risky behaviors.

Speech and language pathologists and physical and occupational therapists can help adjustment to some of the changes caused by FTD.

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What Are Frontotemporal Disorders Causes Symptoms And Treatment

On this page:

Frontotemporal disorders , 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.

How Does Ftd Progress

Frontotemporal dementia: Signs and Symptoms

The progression of symptoms in behavior, language, and/or movement varies by individual, but FTD brings an inevitable decline in functioning. The length of progression varies from 2 to over 20 years.

As the disease progresses, the person affected may experience increasing difficulty in planning or organizing activities. They may behave inappropriately in social or work settings, and have trouble communicating with others, or relating to loved ones.

Over time, FTD predisposes an individual to physical complications such as pneumonia, infection, or injury from a fall. Average life expectancy is 7 to 13 years after the start of symptoms . The most common cause of death is pneumonia.

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What Causes Frontotemporal Dementia

Frontotemporal dementia occurs when abnormal proteins build up in the brain. This leads to death of brain cells and deterioration and shrinkage of the frontal and temporal lobes of the brain. Sometimes the disease is genetic, and passed down through families, but in most cases no specific cause can be found.

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 persons symptoms as well as the results of brain scans and genetic tests.

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Can Lifestyle Changes Help Frontotemporal Dementia

Medication can be effective for treating dementia, but lifestyle treatments can help, too. Helping people find a comfortable environment can help them cope with the symptoms of dementia.

Environment is important. Maintaining an environment that doesnt upset someone is vital. Make sure your home is well-lit and has minimal noise. People with behavior problems need to be in environments that are familiar. They may also need to avoid large crowds.

People with speech problems may need to be in environments where communication is easier. They may wish to keep tools for communicating, like a pen and paper, with them all the time.

What Is The Difference Between Ftd And Alzheimers

Frontotemporal Dementia...What Is It?

Like Alzheimers disease, FTD causes brain atrophy that leads to a progressive loss of brain function. Key differences between the two diseases include:

  • Age at diagnosis: Symptoms of FTD usually appear between the ages of 45 and 65, whereas the majority of Alzheimers cases occur in people over age 65.
  • From behavior changes to memory loss: Changes in behavior are an early sign of FTD, and problems with memory may occur in advanced stages. In contrast, Alzheimers affects memory early on and may lead to behavior issues as it progresses.
  • Speech problems: People with FTD often suffer greater problems speaking, understanding speech and reading than people with Alzheimers.

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What Are The Causes Of Frontotemporal Dementia

The cause of FTD is not known, and may differ between individuals. In about half of FTD patients, a normal brain protein called tau accumulates abnormally and forms deposits. In others, a separate protein known as TDP43 accumulates in the same way.

Unlike other dementias, FTD is highly heritable. Approximately 40% of individuals with FTD have an affected family member. In these individuals, FTD is usually caused by changes in one of their genes.

The diagnosis may be confirmed after death with a brain autopsy. Genetic testing can help reveal an underlying mutation responsible for FTD in about 40% of patients.

What Are The Types Of Frontotemporal Dementia

There are several disorders that can result in frontotemporal dementia, all of which can affect middle-aged and older adults.

  • Behavioral variant frontotemporal dementia is the most common frontotemporal disorder. It causes changes in personality, behavior, emotions, and judgment.
  • Primary progressive aphasia involves changes in the ability to communicate to speak, read, write, and understand what others are saying.
  • Movement disorders happen when the parts of the brain that control movement are affected. There are two types of rare movement disorders associated with FTD: corticobasal syndrome and progressive supranuclear palsy. Other movement-related disorders include frontotemporal dementia with parkinsonism and frontotemporal dementia with amyotrophic lateral sclerosis . Symptoms can vary among these movement disorders.

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Description Of Authors Roles

M. Landqvist Waldö formulated the research questions, designed the study, collected and analyzed all data and wrote the article. L. Gustafson assisted in formulating the research questions, provided support, advice and revised the paper. U. Passant helped to design the study, supervised the clinical data collection and assisted in writing the paper. E. Englund was responsible for the neuropathological material, supervised the project and revised the paper.

Problems With Mental Abilities

What is Frontotemporal Dementia?

Problems with thinking do not tend to occur in the early stages of frontotemporal dementia, but these often develop as the condition progresses.

These can include:

  • difficulty working things out and needing to be told what to do
  • poor planning, judgement and organisation
  • becoming easily distracted
  • thinking in a rigid and inflexible way
  • losing the ability to understand abstract ideas
  • difficulty recognising familiar people or objects
  • memory difficulties, although this is not common early on

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What Are The Signs And Symptoms Of Frontotemporal Dementia

The signs and symptoms of frontotemporal dementia vary from person to person and the order in which they appear can also vary. Changes in the frontal lobe of the brain are generally associated with behavioral symptoms and may also lead to movement symptoms. Changes in the temporal lobe generally lead to disorders of language and emotion.

Symptoms of frontotemporal dementia and associated disorders may include:

Caring For A Loved One

Some tips for keeping the person safe and maximizing their quality of life include:

  • helping them manage their finances and apply for disability payments, if appropriate
  • preempting any physical, social, or psychological risks they may face
  • making adaptations to reduce the risk of falls
  • seeking counseling for depression and anxiety, if applicable
  • maintaining a regular routine
  • helping them avoid triggers of hypersexuality, overeating, and other behaviors that can lead to problems

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The Future Of Ftd Treatment

Researchers are continuing to explore the biological changes in the body, including genetic mutations 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. To find out more, talk to your health care provider or visit the Alzheimers.gov Clinical Trials Finder.

How Is Frontotemporal Dementia Diagnosed

Frontotemporal dementia

Family members are often the first to notice subtle changes in behavior or language skills. Its important to see a healthcare provider as early as possible to discuss:

  • Symptoms, when they began, and how often they occur
  • Medical history and previous medical problems
  • Medical histories of family members
  • Prescription medicines, over-the-counter medicines, and dietary supplements taken

No single test can diagnose FTD. Typically, healthcare providers will order routine blood tests and perform physical exams to rule out other conditions that cause similar symptoms. If they suspect dementia, they may:

  • Evaluate neurological status health including reflexes, muscle strength, muscle tone, sense of touch and sight, coordination, and balance
  • Assess neuropsychological status such as memory, problem-solving ability, attention span and counting skills, and language abilities
  • Order magnetic resonance imaging or computed tomography scans of the brain

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Lack Of Sympathy And Empathy

A person with frontotemporal dementia symptoms puts himself first. He or she has difficulties to see and understand the needs of other people .

They show less personal warmth and love even to his wife or her husband. Besides, a person can show no interest in their children or grandchildren.

Which can be difficult to understand and to accept for family members.

Moreover, a person can be less socially active or doesnt show any interest in the social environment, events or celebrations.

This behavior can make the person appear harmful or selfish and has to be understood as illness and not as an unfriendly attitude or hostility.

Frontotemporal Dementia Causes And Risk Factors

Scientists donât know for sure what causes FTD. But it starts when nerve cells in two parts, or âlobes,â of the brain — the front and the side — die. That causes the lobes to shrink.

Doctors believe FTD may be genetic. About 4 out of 10 people who have it also have a relative who had it or some other form of dementia.

Genetic FTD happens when you have a mutation, or change, in one of three genes:

  • C9ORF72

The type of mutation you have affects the type of symptoms you have.

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What Is The Prognosis For Frontotemporal Dementia

The outcome for people with FTD is poor. The disease progresses steadily and often rapidly, ranging from less than 2 years in some individuals to more than 10 years in others. Eventually some individuals with FTD will need 24-hour care and monitoring at home or in an institutionalized care setting.

Frontotemporal Dementia Risk Factors And Complications

What is frontotemporal dementia?

For most people with FTD, the cause of the disease is unknown.

People with a family history of the disease are more likely to get it. Between 10% to 30% of bvFTD cases are genetic.

There are no known risk factors other than a family history of FTD. Those with this risk of the disease may want to get genetic counseling and testing.

Complications of FTD include:

  • Problems interacting with others .
  • Infections.
  • Pneumonia.

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Managing The Effects Of Frontotemporal Dementia

For the person with dementia:

  • Establish a routine and regular activities
  • Carry a card that lets people know you have dementia in an emergency situation. These can be ordered from our Admiral Nurse Dementia Helpline
  • Try to monitor your food and fluid intake as there can be weight gain due to excessive eating and a fixation with sweet, calorific foods
  • If you experience language difficulties, ask the GP for a Speech and Language Therapist referral so new ways of communicating can be explored

For the family carer:

  • Remember that the changes in personality, inappropriate social behavior and apathy are due to the condition and are not intentional
  • As the person affected may not have insight into the changes experienced, trying to correct the persons behaviour could be resisted and could lead to arguments
  • The family may need specialist advice and support to come to terms with and manage the changes experienced
  • A specialist practitioner such as an Admiral Nurse could also help the family develop strategies for dealing with the changes experienced

What Functions Are Controlled By The Frontal And Temporal Lobe Of The Brain

The frontal lobes control executive functioning, meaning a persons ability to plan in a step-by-step process, to choose priorities, to keep track of several tasks at one time, and make adjustments to ones behavior. The frontal lobe also controls social and emotional responses. In addition, the frontal lobe helps in linking words to form sentences and is crucial making involuntary muscle movements.

The temporal lobes are the major language center, involved with speaking, reading, writing, understanding words and their meanings, and recognizing objects. The temporal lobe also plays a role in recognizing and responding to emotions and is a major memory center.

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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.

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