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How Is Frontotemporal Dementia Causes

What Causes Frontotemporal Dementia

Urvashis story: living with 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.

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 persons 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 , 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.

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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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.

Treatment And Management Of Frontotemporal Dementia

Management of Psychiatric Features in Frontotemporal Dementia

Like Alzheimerâs and most other types of dementia, there are unfortunately no specific treatments that can cure any of the types of frontotemporal dementia. Unlike other types of dementia, there are also no treatments to slow down the progression of FTD. To make matters worse, due to the difficulty of the diagnosis, individuals may go a long time before they get any help in managing their symptoms. With the correct medications, however, behavioral symptoms like irritability, depression, and agitation can be better managed.

Even though medications to manage symptoms can help improve the personâs quality of life, FTD inevitably gets worse over time. As each person with FTD is different, the speed of decline and the manifestation of symptoms will vary. Symptoms of FTD progress at a rapid, steady rate. Individuals with frontotemporal dementia can survive between two to fifteen years and will most likely need 24-hour assistance with their Activities of Daily Living in their later years.

Quick Fact:

Changes in the patientâs behavior are often the first noticeable symptoms in bvFTD, the most common type of FTD.

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What Are The Risk Factors For Frontotemporal Dementia

Frontotemporal dementia has one known risk factor: genetics. Scientists have found several genes related to the disease. If one of your family members is diagnosed with frontotemporal dementia, you have a greater risk.

However, not everyone with a family history will develop a problem. Its also estimated that more than half of the individuals diagnosed with frontotemporal dementia dont have a family history associated with the disease.

Doctors cant diagnose frontotemporal dementia with a single test. Instead, your doctors will try to rule out other conditions or diseases that cause similar symptoms.

Some of the tests used to diagnose frontotemporal dementia include:

  • blood tests: These can help identify other possible causes.
  • neuropsychological testing: These tests check your judgment and memory skills, and help determine what type of dementia you may have.
  • brain imaging: Doctors will check for tumors or blood clots.
  • MRI: A magnetic resonance imaging test gives doctors a detailed image of your brain.
  • CT scan: A computerized tomography scan creates images of your brain in layers.

Frontotemporal dementia cannot be cured. Treatment is aimed at managing and alleviating symptoms.

Common treatments include:

Causes Of Frontotemporal Dementia

Frontotemporal dementia is caused by clumps of abnormal protein forming inside brain cells. These are thought to damage the cells and stop them working properly.

The proteins mainly build up in the frontal and temporal lobes of the brain at the front and sides. These are important for controlling language, behaviour, and the ability to plan and organise.

It’s not fully understood why this happens, but there’s often a genetic link. Around 1 in 8 people who get frontotemporal dementia will have relatives who were also affected by the condition.

If you have a family history of frontotemporal dementia, you may want to consider talking to your doctor about being referred to a geneticist and possibly having a genetic test to see if you’re at risk.

There’s a lot of research being done to try to improve understanding of the causes of frontotemporal dementia so treatments can be discovered.

If you’re interested in helping with research, you can speak to a doctor or register your interest on Join Dementia Research.

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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.

Background On Frontotemporal Dementia

What is Frontotemporal Dementia?

Frontotemporal Dementia is characterized by degeneration of the frontal lobe which lead to behavior and executive function changes. Researchers consider that hereditary factors are important in the aetiology of Frontotemporal Dementia. The matter has been continuously investigated ever since. These investigations showed that children adopted away from their biological parents at birth are at significantly greater risk of later developing Frontotemporal Dementia if there is dementia among their biological relatives, but not if it is present among their adoptive relatives . These findings demonstrate that the enhanced tendency to develop Frontotemporal Dementia in these people is not due to a shared environment but to shared genetic material. One of the major criteria used for distinguishing frontal variant dementia is the existence of executive deficits as shown by performance on frontal tasks . The boundary between normality and Frontotemporal Dementia is not clear-cut. It has been known for a long time that abnormal features can be observed before the onset of a clear-cut psychotic episode, and so it has generally been assumed that these transient or partial forms would always evolve into clear-cut and sustained psychotic features. However, this is not always the case.

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Subtypes And Related Disorders

The main subtypes of frontotemporal dementia are behavioral variant FTD, semantic dementia, progressive nonfluent aphasia, and FTD associated with amyotrophic lateral sclerosis . Two distinct rare subtypes are neuronal intermediate filament inclusion disease, and basophilic inclusion body disease. Related disorders are corticobasal syndrome, and progressive supranuclear palsy.

What Is Frontotemporal Dementia

Frontotemporal dementia , a common cause of dementia, is a group of disorders that occur when nerve cells in the frontal and temporal lobes of the brain are lost. This causes the lobes to shrink. FTD can affect behavior, personality, language, and movement.

These disorders are among the most common dementias that strike at younger ages. Symptoms typically start between the ages of 40 and 65, but FTD can strike young adults and those who are older. FTD affects men and women equally.

The most common types of FTD are:

  • Frontal variant. This form of FTD affects behavior and personality.
  • Primary progressive aphasia. Aphasia means difficulty communicating. This form has two subtypes:
  • Progressive nonfluent aphasia, which affects the ability to speak.
  • Semantic dementia, which affects the ability to use and understand language.

A less common form of FTD affects movement, causing symptoms similar to Parkinson disease or amyotrophic lateral sclerosis .

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Be A Part Of Finding Better Treatments

Do you or a family member have frontotemporal dementia? Researchers are looking for people like you to participate in a study to track disease progression and advance treatments the ARTFL-LEFFTDS Longitudinal Frontotemporal Lobar Degeneration Research Consortium.

Can Lifestyle Changes Help Frontotemporal Dementia

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.

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Can We Predict Molecular Pathologies In Frontotemporal Dementia

Despite recent histopathological and genetic advances,24 accurate prediction of underlying molecular pathologies within the FTD spectrum remains problematic . This has important implications for targeting future disease modifying therapies and, more immediately, for counselling of patients and family members. While phenotypic variation and overlap are substantial, certain clinico-anatomical profiles are consistently associated with particular molecular pathologies .21 These include FTD with motor neurone disease associated with TDP-43 pathology the semantic dementia syndrome and focal, asymmetric temporal lobe atrophy with TDP-43 type C pathology behavioural variant FTD of very young onset and prominent caudate atrophy with FUS pathology and the progressive supranuclear palsy syndrome with a specific tauopathy. Non-fluent speech breakdown and the development of parkinsonism are more commonly associated with tau than non-tau pathologies. The heritability of particular FTD syndromes varies substantially3: semantic dementia is generally sporadic, whereas behavioural variant FTD and corticobasal syndrome are highly genetically as well as pathologically heterogeneous. Useful neuroanatomical associations which may help guide genetic testing include relatively symmetric anteromesial temporal lobe atrophy with MAPT mutations and striking asymmetric hemispheric atrophy with GRN mutations .4

What Is The Prognosis For Someone With Frontotemporal Dementia

Frontotemporal dementia worsens over time. Eventually people with FTD will need help with their activities of everyday living. Some people may need 24-hour care at home or at living facilities or nursing homes. The speed of decline varies from person to person, but the disease course typically ranges from two to 10 years.

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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.

Are There Options For Treatment

Frontotemporal dementia: Signs and Symptoms

Today, there is no cure for FTD. Unfortunately, no current treatments slow or stop the progression of the disease. However, if you or a family member or loved one are affected, there are important steps that you can take to preserve and maximize quality of life. A growing number of interventions not limited to medication can help with managing FTD symptoms.

It is important for you and your care partner to identify a team of experts who can help with coordinating care and with the legal, financial, and emotional challenges brought on by this disease.

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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 : behavioral variant frontotemporal dementia , primary progressive aphasia , and movement disorders.

How Ftd And Alzheimer’s Differ

In Alzheimers, the typical initial symptoms are short-term memory impairment and difficulty learning something new. In FTD, the memory usually remains intact initially early symptoms include difficulty with appropriate social interactions and emotions, as well as some language challenges.

FTD and Alzheimers also differ in how the brain is physically affected. FTD mainly affects the brain’s frontal and temporal lobes, whereas Alzheimers affects the temporal lobes in early stages, but with progression impacts most areas of the brain.

FTD also targets younger individuals. The average age of onset for FTD is about 60 years old. While some people have early-onset Alzheimers, the majority of patients are over 65 and many of those are well into their 70s or 80s.

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What Is The Difference Between Ftd And Alzheimers

There are key differences between FTD and Alzheimers. It is important to understand the differences so there is an accurate diagnosis for patients.

While Alzheimers risk goes up as people age, people are often diagnosed with FTD in their 40s and early 60s. The Alzheimers Association reports that with Alzheimers, memory loss is often the first symptom that appears. With FTD, people usually experience behavioral and language changes first. In addition, people with Alzheimers may have difficulty recalling names, but usually dont have serious problems with speech until later stages. In the early stages of FTD, someone may be difficult to understand or have trouble reading. As Alzheimers progresses, hallucinations become more common, though they are not as common in FTD.

Frontotemporal Dementia Risk Factors

Frontotemporal dementia: symptoms, diagnosis and treatment

Generally, you are at higher risk of having frontotemporal dementia if you have a family history of dementia. Scientists estimate that one in ten cases of FTD are inherited. There are some genes which give an increased risk of inherited forms of FTD.

Other risk factors for FTD are less well- understood. Researchers have found correlations between obesity and frequent smoking to an increased chance of having FTD. Having diabetes, a prior head injury or an autoimmune disorder like rheumatoid arthritis are also understood to be risk factors for FTD.

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How Does Ftd Progress

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.

Causes Of Frontotemporal Disorders

In frontotemporal dementia, the frontal and temporal lobes of the brain shrink or atrophy. Typically, the cause is unknown. Certain genetic mutations have been linked to the development of specific subtypes of FTD, but more than 50% of individuals who develop FTD have no family history of the disease. That being said, other than family history of the disease, there are not currently any known risk factors for developing FTD.

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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.

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