- By Susan Barber Lindquist What’s frontotemporal dementia?
- February 23, 2024
An unusual group of problems could trigger adjustments in persona, conduct, language, motion
Frontotemporal dementia (FTD) is a gaggle of neurologic problems related to adjustments in persona, conduct, language or motion. Some FTD varieties are inherited, and a few will not be. Usually, folks develop FTD signs earlier than age 60.
Whereas there is no such thing as a treatment for this progressive decline, FTD analysis has made vital strides prior to now 20 years, says Dr. Bradley Boeve (boh-VAY), a Mayo Clinic neurologist.
“There are few different neurodegenerative problems the place we have now realized a lot over a comparatively quick time frame,” says Dr. Boeve, a co-principal investigator of the ALLFTD Examine, a world consortium funded by the Nationwide Institutes of Well being to focus on FTD.
“We perceive the biology of the illness that significantly better. The optimism for therapeutics is a lot better than even two or three years in the past. There’s undoubtedly hope.”
Watch: Dr. Bradley Boeve talks about frontotemporal degeneration
https://youtube.com/watch?v=-rEhlbyqm0Qpercent3Ffeaturepercent3Doembedpercent26enablejsapipercent3D1
Journalists: Broadcast-quality video is accessible within the downloads on the finish of the submit. Please courtesy: Mayo Clinic Information Community. Title tremendous/CG: Bradley Boeve,M.D./Neurology/Mayo Clinic.
What are the varieties of FTD problems?
Because the identify suggests, FTD impacts the frontal lobe and/or temporal lobes of the mind. Indicators 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.
A number of problems fall beneath the umbrella time period of frontotemporal degeneration.
- Frontotemporal dementia (often known as behavioral variant frontotemporal dementia): Mind adjustments, primarily within the frontal lobe, trigger the persona and conduct adjustments of frontotemporal dementia. Widespread indicators are:
- More and more inappropriate social conduct, Lack of empathy and different folks expertise, akin to having sensitivity to a different’s emotions
- Lack of judgment
- Lack of inhibition
- Lack of curiosity (apathy), which may be mistaken for melancholy
- Repetitive compulsive conduct, akin to tapping, clapping or smacking lips
- Decline in private hygiene Adjustments in consuming habits, normally overeating or preferring sweets and carbohydrates
“Persona (and) conduct adjustments are inclined to happen early. Disinhibited conduct — doing issues which can be a bit off-color or out of character for that particular person,” Dr. Boeve explains. “Lack of empathy, for instance, if somebody had been to be at a funeral, not exhibiting the same old varieties of sorrow for such a somber setting.”
- Main progressive aphasia: Folks lose the power to talk and write, and/or perceive written or spoken language. Main progressive aphasia additionally has subtypes.
“Some folks have difficulties expressing language,” Dr. Boeve explains. “They comprehend moderately nicely, however getting the lips and the tongue to control in order that phrases come out with fluency is extra of a problem. Versus one other variant, developing with the names of individuals or objects is the first issue. (For) others, it’s a little bit of a combination — comprehending, additionally expressing in addition to naming objects.”
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. Within the U.S., behavioral variant frontotemporal dementia and first progressive aphasia are estimated to have an effect on 50,000-60,000 folks.
Different 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 point or slurred speech usually are the primary indicators.
- Corticobasal syndrome ends in poor coordination and stiffness within the arms or legs (usually worse on one aspect of the physique than the opposite), issue considering, and bother with speech and language.
- Progressive supranuclear palsy causes issues with strolling, steadiness, eye actions and swallowing.
If FTD problems have so many similarities, how are they recognized?
Figuring out a prognosis may be difficult. Sufferers with behavioral variant frontotemporal dementia typically are misdiagnosed with a psychiatric or stress-related sickness, akin to atypical melancholy, late-onset bipolar dysfunction or schizophrenia, and even midlife disaster, Dr. Boeve says.
With help from checks to assist rule out different problems, a health care provider diagnoses a affected person based mostly on signs. For instance, if sufferers are having extra conduct and persona adjustments — with or with none language or motor issues — if they’ve bother with problem-solving and judgment, however reminiscence is comparatively preserved, that’s extra typical of behavioral variant frontotemporal dementia, Dr. Boeve says.
Mind imaging checks are essential. An MRI scan could present shrinking of the frontal or temporal lobes of the mind. A PET scan could present metabolic adjustments in these similar 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 people with FTD have a genetic trigger or contributor, and that’s extra widespread than in Alzheimer’s illness, Lewy physique illness and different neurologic problems,” says Dr. Boeve, The Little Household Basis Professor of Lewy Physique Dementia.
Main progressive aphasia usually may be recognized with language-based testing and mind imaging research. Corticobasal syndrome and progressive supranuclear palsy may be recognized with a neurologic examination and mind imaging research.
For ALS, different checks akin to 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 line with the Affiliation for Frontotemporal Degeneration (AFTD). “We regularly name 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 sluggish FTD, however scientific trials are ongoing. Immediately, therapy focuses on managing signs.
For behavioral variant frontotemporal dementia, for instance, antidepressant and antipsychotic medicines could assist scale back conduct issues, however all medicine have unintended effects, so that they should be thought-about with warning. Medicines used for Alzheimer’s illness will not be efficient, which is another excuse an correct prognosis is essential, Dr. Boeve notes.
“The mainstay of remedy is nonmedication approaches — learn how to help the affected person and household to handle the disinhibition or the repetitive conduct, particularly when in social settings or in public,” Dr. Boeve says. “It’s fairly difficult for households, however these methods may be useful.”
Nonmedication methods embrace:
- Avoiding actions that set off the undesirable conduct.
- Sustaining a relaxed atmosphere.
- Offering structured routines.
- Simplifying every day duties.
- Distracting or redirecting consideration from drawback behaviors.
For language issues, speech remedy could also be useful. For motor problems, bodily and occupational remedy, together with structured workouts, could assist power, mobility and steadiness.
How is frontotemporal dementia completely different from Alzheimer’s illness?
Alzheimer’s illness is extra widespread amongst folks 75 and older. Nonetheless, folks with early onset Alzheimer’s or frontotemporal dementia usually begin exhibiting signs in midlife, from roughly age 30 to 60. Reminiscence adjustments are much less widespread 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 these proteins which can be very useful to strongly help underlying Alzheimer’s illness or strongly argue towards underlying Alzheimer’s illness,” Dr. Boeve says. “We don’t have these varieties of fluid biomarkers based mostly on blood testing or cerebrospinal fluid but for FTD, though that’s an energetic space of analysis.”
What sources can be found?
Assist teams and medical associations for the assorted frontotemporal degeneration problems can assist sufferers, their households and care companions cope.
“Establishing an early and correct prognosis of FTD is the primary key step,” Dr. Boeve says. “Then sufferers and their households can develop a complete administration program with their well being care staff. Engagement with different sources and nationwide organizations dedicated to FTD care and analysis can present further strategies to face the challenges forward.”
