Www.nia.hih.gov Frontotemporal Disorders Information for Patients Families and Friends

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

FTD is progressive, meaning symptoms go worse over time. In the early stages, people may have just one symptom. As the affliction progresses, other symptoms appear as more parts of the encephalon are afflicted. Information technology is difficult to predict how long someone with FTD will alive. Some people live more 10 years after diagnosis, while others live less than two years later they are diagnosed.

There is currently no cure for FTD, and no treatments irksome or stop the progression of the affliction, but at that place are means to help manage the symptoms.

What do the terms hateful?

Share this infographic and assist spread the word well-nigh understanding unlike types of dementia.

I of the challenges shared by people living with these disorders, families, clinicians, and researchers is what terminology to utilize. Here, nosotros take used the term frontotemporal disorders to narrate this grouping of diseases and the abbreviation FTD, which is normally used to refer to them. Other terms used include frontotemporal lobar degeneration and frontotemporal dementia, just it'due south important to notation that with some frontotemporal disorders, the main symptoms are issues with speech or motility, 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 on stages, it can be hard to know which blazon of FTD a person has considering symptoms and the order in which they appear can vary from one person to another. Too, the same symptoms can appear across different disorders and vary from i stage of the disease to the next as different parts of the brain are afflicted.

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

In that location are three types of frontotemporal disorders (FTD): behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and motion disorders.

Behavioral variant frontotemporal dementia

The about mutual FTD, bvFTD, involves changes in personality, behavior, and judgment. People with this disorder may have problems with noesis, only their retentivity may stay relatively intact. Symptoms can include:

• Problems planning and sequencing (thinking through which steps come starting time, 2d, then on)
• Difficulty prioritizing tasks or activities
• Repeating the same activity or maxim the same give-and-take over and over
• Acting impulsively or proverb or doing inappropriate things without considering how others perceive the behavior
• Becoming disinterested in family unit or activities they used to intendance about

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

Master progressive aphasia

PPA involves changes in the ability to communicate — to use language to speak, read, write, and sympathise what others are saying. This includes difficulty using or understanding words (aphasia) and difficulty speaking properly (e.g., slurred speech communication). 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 non apparent at first but can develop over fourth dimension. In addition, some people with PPA may feel meaning behavioral changes, similar to those seen in bvFTD, equally the disease progresses.

At that place 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 than trouble speaking and may omit words that link nouns and verbs (such every bit to, from, the). Somewhen, 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 problem finding the right words during a chat but can understand words and sentences. The person does not accept problems with grammar.

Researchers do not fully understand the biological ground of the different types of PPA. But they promise ane day to link specific linguistic communication problems with the changes in the brain that cause them.

Move disorders

Two rare neurological movement disorders associated with FTD, corticobasal syndrome and progressive supranuclear palsy, occur when the parts of the encephalon that command movement are affected. The disorders may bear upon thinking and language abilities, also.

• Corticobasal syndrome tin can be caused past corticobasal degeneration — a gradual cloudburst (shrinkage) and loss of nerve cells in specific parts of the brain. This degeneration causes progressive loss of the ability to control move, typically starting time effectually age sixty. The well-nigh prominent symptom may be apraxia, the disability to utilise the easily 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 showtime on 1 side of the body, but eventually both sides are affected. Occasionally, a person with corticobasal syndrome starting time has language bug or problem orienting objects in space and afterward develops motility symptoms. Non everyone who has corticobasal syndrome has issues with memory, cognition, language, or behavior.
• Progressive supranuclear palsy causes issues with balance and walking. People with the disorder typically move slowly, experience unexplained falls, lose facial expression, and have torso stiffness, particularly in the cervix and upper trunk — symptoms similar to those of Parkinson's disease. A hallmark sign of this disorder is problem with centre movements, particularly looking downwards. These symptoms may give the face a fixed stare. Problems with beliefs, 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 tin be an inherited affliction acquired by a genetic tau mutation. Symptoms include movement issues like to those of Parkinson's illness, such as slowed motility, stiffness, and balance issues, and changes in beliefs or linguistic communication.
• FTD-ALS, too 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 musculus weakness seen in ALS, fine jerks, and fluctuant in muscles. Symptoms of either disease may announced first, with other symptoms developing over time. Mutations in certain genes accept been found in some people with FTD-ALS, though most cases are not hereditary.

What causes FTD?

Scientists are beginning to sympathise the biological and genetic basis for the changes observed in brain cells that atomic number 82 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 torso and help cells function properly. When the proteins don't work properly, for reasons not however fully understood, neurons in specific brain regions are damaged.

In most cases, the crusade of a FTD is unknown. Individuals with a family history of FTD are more than probable to develop such a disorder. About 10 to thirty% of bvFTD is due to specific genetic causes.

FTD that runs in a family is often related to mutations (permanent changes) in certain genes. Genes are basic units of heredity that tell cells how to make the proteins the torso needs to function. Even small changes in a gene may produce an abnormal poly peptide, which tin atomic number 82 to changes in the encephalon and, somewhen, disease.

Scientists have discovered several different genes that, when mutated, can lead to FTD:

• Tau gene (too called the MAPT gene) — A mutation in this gene causes abnormalities in a protein chosen tau, which then forms tangles within neurons and ultimately leads to the destruction of brain cells. Inheriting a mutation in this gene means a person volition almost surely develop a frontotemporal disorder, commonly bvFTD, just the exact age of onset and symptoms cannot be predicted.
• GRN gene — A mutation in this factor can lead to lower production of the protein progranulin, which in turn causes another protein, TDP-43, to go awry in encephalon cells. Many frontotemporal disorders can result, though bvFTD is the most mutual. The GRN gene can crusade dissimilar symptoms in different family members and crusade the disease to brainstorm at different ages.
• C9ORF72 gene — An unusual mutation in this gene appears to be the about common genetic abnormality in familial frontotemporal disorders and familial ALS. This mutation can crusade a frontotemporal disorder, ALS, or both conditions.

In recent years researchers have discovered several other genetic mutations in genes that pb to rare familial types of frontotemporal disorders. These other mutations business relationship for less than 5% of all cases of FTD.

Families afflicted by inherited and familial forms of FTD can help scientists advance inquiry past participating in clinical studies and trials. For more than information, talk with a health care professional or visit the Alzheimers.gov Clinical Trials Finder.

How is FTD diagnosed?

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

To help diagnose frontotemporal dementia, a md may:

• Perform an test and ask most symptoms
• Look at personal and family medical history
• Use laboratory tests to help rule out other conditions
• Order genetic testing
• Carry tests to assess memory, thinking, language skills, and physical performance
• Gild imaging of the brain

A psychiatric evaluation can assist decide if low or another mental wellness condition is causing or contributing to the condition. Only genetic tests in familial cases or a encephalon dissection after a person dies tin ostend a diagnosis of FTD.

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

Treatment and direction of FTD

So far, at that place is no cure for FTD and no mode to slow down or prevent these diseases. However, there are ways to manage symptoms. A squad of specialists — doctors, nurses, and speech, concrete, and occupational therapists — familiar with these disorders can help guide handling.

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 reply can reduce frustration and help provide the best intendance for a person with FTD.

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

• Attempt 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, exist equally sensitive every bit 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 aloofness, limit choices and offer specific choices. Open-ended questions, such equally "What do you want to practise today?" are more difficult to answer than specific ones, such equally "Do y'all want to go to the park or for a walk?".
• Maintain a regular schedule, reduce distractions, and alter the environs to reduce confusion and amend the person's sleep.
• If compulsive eating is an issue, consider supervising eating, limiting nutrient choices, locking cabinets and the refrigerator, and distracting the person with other activities.

To ensure the condom of a person and his or her family unit, caregivers may have to take on new responsibilities or arrange care that was non 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 accept depression doses of antipsychotic medications. If a item medication is not working, a doctor may endeavour another. E'er consult a doc before irresolute, adding, or stopping a drug or supplement.

Treating linguistic communication problems in FTD

Handling of PPA has two goals — maintaining linguistic communication skills and using new tools and other ways to communicate. Handling tailored to a person's specific language trouble and phase of PPA generally works best. Since language power declines over time, different strategies may be needed as the illness progresses. The post-obit strategies may help:

• Use a advice 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 calculator or phone to point to.
• Speak slowly and conspicuously, apply unproblematic sentences, await for responses, and ask for description if needed.
• Piece of work with a speech communication-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 motility problems in FTD

Medications and concrete and occupational therapy may provide pocket-size relief for the move 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 comeback. Physical and occupational therapy may help the person move more easily. Speech therapy can assist them manage language symptoms.

For people with progressive supranuclear palsy, sometimes Parkinson'south disease drugs provide temporary relief for slowness, stiffness, and balance problems. Exercises tin can keep the joints limber, and weighted walking aids — such as a walker with sandbags over the lower front end rung — tin help maintain balance. Speech, vision, and swallowing difficulties usually practice not respond to whatsoever drug treatment. Antidepressants take shown modest success. For people with abnormal center movements, bifocals or special glasses called prisms are sometimes prescribed.

People with FTD-ALS typically pass up quickly over ii to three years. During this time, physical therapy can help treat muscle symptoms, and a walker or wheelchair may be useful. Voice communication therapy may help a person speak more clearly at commencement. Later on, other ways of communicating, such equally a speech synthesizer, can be used. The ALS symptoms of the disorder ultimately make information technology 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 weather tin ensure that people with movement disorders get advisable medical treatment and that their caregivers can aid them live too equally possible.

The future of FTD treatment

Researchers are continuing to explore the biological changes in the body, including genetic mutations and proteins, that pb to FTD and identify and exam possible new drugs and other treatments. They are also developing meliorate ways to track disease progression, then that treatments, when they go bachelor, can exist 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.

Where to observe FTD diagnosis and handling

Columbia-Presbyterian Medical Center
Department of Neurology
New York, NY
646-426-3876

Houston Methodist Hospital
Frontotemporal Degeneration Unit
Houston, TX
713-441-7650

Indiana University Schoolhouse of Medicine
Indiana Alzheimer'southward Disease Center
Indianapolis, IN
317-963-5500

Johns Hopkins Academy School of Medicine
Frontotemporal Dementia and Young-Onset Dementias Clinic
Baltimore, Physician
410-955-5147

Massachusetts General Infirmary
Frontotemporal Disorders Unit
Boston, MA
617-726-1728

Mayo Clinic
Department of Neurology
Rochester, MN
507-538-3270
Jacksonville, FL
904-953-0853
Phoenix or Scottsdale, AZ
800-446-2279

Northwestern University Feinberg School of Medicine
Mesulam Centre for Cognitive Neurology and Alzheimer's Disease
Chicago, IL
312-908-9339

University of Alabama, Birmingham
Neurology Department, Division of Retention Disorders
Birmingham, AL
205-996-3679

Academy of California, Los Angeles
Neurobehavior Clinic
Los Angeles, CA
310-794-1195

Academy of California, San Diego
Shiley-Marcos Alzheimer'southward Illness Research Centre
La Jolla, CA
858-822-4800

Academy of California, San Francisco
Memory and Aging Center
San Francisco, CA
415-353-2057

Academy of Pennsylvania Health System
Penn Frontotemporal Degeneration Centre
Philadelphia, PA
215-349-5863

Washington University
Department of Neurology
St. Louis, MO
314-362-1408

For more information nigh FTD

NIA Alzheimer's and related Dementias Didactics and Referral (ADEAR) Center
800-438-4380
adear@nia.nih.gov
world wide web.nia.nih.gov/alzheimers
The NIA ADEAR Middle offers information and free print publications about Alzheimer's and related dementias for families, caregivers, and health professionals. ADEAR Center staff answer phone, email, and written requests and make referrals to local and national resource.

Alzheimers.gov
www.alzheimers.gov
Explore the Alzheimers.gov portal for information and resources on Alzheimer'southward and related dementias from beyond the federal government.

This content is provided by the NIH National Institute on Aging (NIA). NIA scientists and other experts review this content to ensure it is accurate and up to date.

Content reviewed: July 30, 2021

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Source: https://www.nia.nih.gov/health/what-are-frontotemporal-disorders

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