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What is frontotemporal dementia? – Mayo Clinic News Network

An unusual group of problems could trigger modifications in character, habits, language, motion

Frontotemporal dementia (FTD) is a bunch of neurologic problems related to modifications in character, habits, language or motion. Some FTD varieties are inherited, and a few will not be. Typically, individuals develop FTD signs earlier than age 60.

While there is no treatment for this progressive decline, FTD analysis has made important strides previously twenty years, says Dr. Bradley Boeve (boh-VAY), a Mayo Clinic neurologist.

“There are few other neurodegenerative disorders where we have learned so much over a relatively short period of time,” says Dr. Boeve, a co-principal investigator of the ALLFTD Study, a global consortium funded by the National Institutes of Health to focus on FTD.

“We understand the biology of the disease that much better. The optimism for therapeutics is so much greater than even two or three years ago. There’s definitely hope.”

Watch: Dr. Bradley Boeve talks about frontotemporal degeneration

Journalists: Broadcast-quality video is out there within the downloads on the finish of the publish. Please courtesy: Mayo Clinic News Network. Name tremendous/CG: Bradley Boeve,M.D./Neurology/Mayo Clinic.

What are the forms of FTD problems?

As the title suggests, FTD impacts the frontal lobe and/or temporal lobes of the mind. Signs and signs range, relying on which a part of the mind is atrophying, or shrinking. Genetic mutations and dysfunctional proteins within the mind have been linked to FTD.

Several problems fall beneath the umbrella time period of frontotemporal degeneration.

  • Frontotemporal dementia (often known as behavioral variant frontotemporal dementia): Brain modifications, primarily within the frontal lobe, trigger the character and habits modifications of frontotemporal dementia. Common indicators are:
    • Increasingly inappropriate social habits, Loss of empathy and different individuals expertise, reminiscent of having sensitivity to a different’s emotions
    • Lack of judgment
    • Loss of inhibition
    • Lack of curiosity (apathy), which could be mistaken for depression
    • Repetitive compulsive habits, reminiscent of tapping, clapping or smacking lips
    • Decline in private hygiene Changes in eating habits, normally overeating or preferring sweets and carbohydrates

“Personality (and) behavior changes tend to occur early. Disinhibited behavior — doing things that are a bit off-color or out of character for that individual,” Dr. Boeve explains. “Loss of empathy, for example, if someone were to be at a funeral, not showing the usual types of sorrow for such a somber setting.”

  • Primary progressive aphasia: People lose the flexibility to talk and write, and/or perceive written or spoken language. Primary progressive aphasia additionally has subtypes.

“Some people have difficulties expressing language,” Dr. Boeve explains. “They comprehend reasonably well, but getting the lips and the tongue to manipulate so that words come out with fluency is more of a challenge. As opposed to another variant, coming up with the names of people or objects is the primary difficulty. (For) others, it’s a bit of a mix — comprehending, also expressing as well as naming items.”

The prevalence of behavioral variant frontotemporal dementia and first progressive aphasia subtypes of FTD worldwide is unsure. Estimates are between 15 to 22 per 100,000 people who find themselves 45 to 64 years previous have FTD. In the U.S., behavioral variant frontotemporal dementia and first progressive aphasia are estimated to have an effect on 50,000-60,000 individuals.

Other problems are also grouped beneath the FTD umbrella:

  • Amyotrophic lateral sclerosis, additionally referred to as Lou Gehrig’s illness, causes lack of muscle management. Muscle twitching and limb weak spot or slurred speech usually are the primary indicators.
  • Corticobasal syndrome leads to poor coordination and stiffness within the arms or legs (usually worse on one aspect of the body than the opposite), problem considering, and hassle with speech and language.

If FTD problems have so many similarities, how are they recognized?

Determining a prognosis could be difficult. Patients with behavioral variant frontotemporal dementia generally are misdiagnosed with a psychiatric or stress-associated sickness, reminiscent of atypical depression, late-onset bipolar dysfunction or schizophrenia, and even midlife disaster, Dr. Boeve says.

With help from assessments to assist rule out different problems, a health care provider diagnoses a affected person primarily based on signs. For instance, if sufferers are having extra habits and character modifications — with or with none language or motor issues — if they’ve hassle with drawback-fixing and judgment, however reminiscence is comparatively preserved, that is extra typical of behavioral variant frontotemporal dementia, Dr. Boeve says.

Brain imaging assessments are necessary. An MRI scan could present shrinking of the frontal or temporal lobes of the mind. A PET scan could present metabolic modifications in these identical areas of the mind.

Genetic testing additionally is useful. For instance, the identical mutated gene could trigger a brother to have frontotemporal dementia and a sister to have ALS, Dr. Boeve says.

“Roughly 20% to 40% of individuals with FTD have a genetic cause or contributor, and that is more common than in Alzheimer’s disease, Lewy body disease and other neurologic disorders,” says Dr. Boeve, The Little Family Foundation Professor of Lewy Body Dementia.

Primary progressive aphasia usually could be recognized with language-primarily based testing and mind imaging research. Corticobasal syndrome and progressive supranuclear palsy could be recognized with a neurologic examination and mind imaging research.

For ALS, different assessments reminiscent of an electromyogram, or EMG, along with a affected person’s signs and scans, could assist decide a prognosis.

On common, it takes greater than three years to get an correct prognosis, in response to the Association for Frontotemporal Degeneration (AFTD). “We often call this the diagnostic odyssey of FTD,” says Dr. Boeve, a member of the AFTD Medical Advisory Council.

What therapies can be found?

No therapies can be found to cease or gradual FTD, however scientific trials are ongoing. Today, remedy focuses on managing signs.

For behavioral variant frontotemporal dementia, for instance, antidepressant and antipsychotic drugs could assist scale back habits issues, however all medicine have unwanted effects, in order that they have to be thought of with warning. Medications used for Alzheimer’s illness will not be efficient, which is one more reason an correct prognosis is necessary, Dr. Boeve notes.

“The mainstay of therapy is nonmedication approaches — how to assist the patient and family to manage the disinhibition or the repetitive behavior, especially when in social settings or in public,” Dr. Boeve says. “It’s quite challenging for families, but those strategies can be helpful.”

Nonmedication methods embrace:

  • Avoiding actions that set off the undesirable habits.
  • Maintaining a relaxed atmosphere.
  • Providing structured routines.
  • Simplifying day by day duties.
  • Distracting or redirecting attention from drawback behaviors.

For language issues, speech remedy could also be useful. For motor problems, bodily and occupational remedy, together with structured exercises, could assist strength, mobility and stability.

How is frontotemporal dementia completely different from Alzheimer’s illness?

Alzheimer’s disease is extra frequent amongst individuals 75 and older. However, individuals with early onset Alzheimer’s or frontotemporal dementia usually start exhibiting signs in midlife, from roughly age 30 to 60. Memory modifications are much less frequent with frontotemporal dementia than with Alzheimer’s illness.

In FTD, dysfunctional proteins within the mind, referred to as tau or TDP-43, could also be drivers of illness. In Alzheimer’s illness, amyloid and tau are the 2 dysfunctional proteins, and they are often measured in a affected person’s spinal fluid. The type of irregular tau that may be concerned in FTD is completely different than in Alzheimer’s illness, which provides to the complexity of prognosis when utilizing biomarkers, Dr. Boeve says.

“There’s a profile of those proteins that are very helpful to strongly support underlying Alzheimer’s disease or strongly argue against underlying Alzheimer’s disease,” Dr. Boeve says. “We don’t have those types of fluid biomarkers based on blood testing or cerebrospinal fluid yet for FTD, although that’s an active area of research.”

What assets can be found?

Support teams and medical associations for the assorted frontotemporal degeneration problems can assist sufferers, their households and care companions cope.

“Establishing an early and accurate diagnosis of FTD is the first key step,” Dr. Boeve says. “Then patients and their families can develop a comprehensive management program with their health care team. Engagement with other resources and national organizations committed to FTD care and research can provide additional methods to face the challenges ahead.”


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