Learn About Frontotemporal Dementia

What is the definition of Frontotemporal Dementia?

Frontotemporal dementia (FTD) is a rare form of dementia that is similar to Alzheimer disease, except that it tends to affect only certain areas of the brain.

Dementia may also be referred to as major neurocognitive disorder.

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What are the alternative names for Frontotemporal Dementia?

Semantic dementia; Dementia - semantic; Frontotemporal dementia; FTD; Arnold Pick disease; Pick disease; 3R tauopathy; Frontotemporal lobar degeneration (FTLD)

What are the causes of Frontotemporal Dementia?

People with FTD have abnormal substances (called tangles, Pick bodies, Pick cells, and tau proteins) inside nerve cells in the damaged areas of the brain.

The exact cause of the abnormal substances is unknown. Many different abnormal genes have been found that can cause FTD. Some cases of FTD are passed down through families.

FTD is rare. It can occur in people as young as 20. But it usually begins between ages 40 and 60. The average age at which it begins is 54.

What are the symptoms of Frontotemporal Dementia?

The disease gets worse slowly. Tissues in parts of the brain shrink over time. Symptoms such as behavior changes, speech difficulty, and problems thinking occur slowly and get worse.

Early personality changes can help doctors tell FTD apart from Alzheimer disease. (Memory loss is often the main, and earliest, symptom of Alzheimer disease.)

People with FTD tend to behave the wrong way in different social settings. The changes in behavior continue to get worse and are often one of the most disturbing symptoms of the disease. Some persons have more difficulty with decision-making, complex tasks, or language (trouble finding or understanding words or writing). There are variations of FTD seen with other nervous system problems such as:

  • Amyotrophic Lateral Sclerosis (ALS/Lou Gehrig disease)
  • Primary progressive aphasia
  • Progressive supranuclear palsy

General symptoms include:

BEHAVIORAL CHANGES:

  • Not able to keep a job
  • Compulsive behaviors
  • Impulsive or inappropriate behavior
  • Inability to function or interact in social or personal situations
  • Problems with personal hygiene
  • Repetitive behavior
  • Withdrawal from social interaction

EMOTIONAL CHANGES

  • Abrupt mood changes
  • Decreased interest in daily living activities
  • Failure to recognize changes in behavior
  • Failure to show emotional warmth, concern, empathy, sympathy
  • Inappropriate mood
  • Not caring about events or environment

LANGUAGE CHANGES

  • Cannot speak (mutism)
  • Decreased ability to read or write
  • Difficulty finding a word
  • Difficulty speaking or understanding speech (aphasia)
  • Repeating anything spoken to them (echolalia)
  • Shrinking vocabulary
  • Weak, uncoordinated speech sounds

NERVOUS SYSTEM PROBLEMS

  • Increased muscle tone (rigidity)
  • Memory loss that gets worse
  • Movement/coordination difficulties (apraxia)
  • Weakness

OTHER PROBLEMS

  • Urinary incontinence
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What are the current treatments for Frontotemporal Dementia?

There is no specific treatment for FTD. Medicines may help manage mood swings.

Sometimes, people with FTD take the same medicines used to treat other types of dementia.

In some cases, stopping or changing medicines that worsen confusion or that are not needed can improve thinking and other mental functions. Medicines include:

  • Analgesics
  • Anticholinergics
  • Central nervous system depressants
  • Cimetidine
  • Lidocaine

It is important to treat any disorders that can cause confusion. These include:

  • Anemia
  • Decreased oxygen (hypoxia) level
  • Heart failure
  • High carbon dioxide level
  • Infections
  • Kidney failure
  • Liver failure
  • Nutritional disorders
  • Thyroid disorders
  • Mood disorders, such as depression

Medicines may be needed to control aggressive, dangerous, or agitated behaviors.

Behavior modification can help some people control unacceptable or dangerous behaviors. This consists of rewarding appropriate or positive behaviors and ignoring inappropriate behaviors (when it is safe to do so).

Talk therapy (psychotherapy) does not always work. This is because it can cause further confusion or disorientation.

Reality orientation, which reinforces environmental and other cues, may help reduce disorientation.

Depending on the symptoms and severity of the disease, monitoring and help with personal hygiene and self-care may be needed. Eventually, there may be a need for 24-hour care and monitoring at home or in a special facility. Family counseling can help the person cope with the changes needed for home care.

Care may include:

  • Adult protective services
  • Community resources
  • Homemakers
  • Visiting nurses or aides
  • Volunteer services

People with FTD and their family may need to seek legal advice early in the course of the disorder. Advance care directive, power of attorney, and other legal actions can make it easier to make decisions regarding the care of the person with FTD.

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What are the support groups for Frontotemporal Dementia?

You can ease the stress of FTD by joining a support group. Sharing with others who have common experiences and problems can help you not feel alone. More information and support for people with FTD and their families can be found at:

The Association for Frontotemporal Degeneration -- www.theaftd.org/get-involved/in-your-region/

What is the outlook (prognosis) for Frontotemporal Dementia?

The disorder quickly and steadily becomes worse. The person becomes totally disabled early in the course of the disease.

FTD commonly causes death within 8 to 10 years, usually from infection, or sometimes because body systems fail.

When should I contact a medical professional for Frontotemporal Dementia?

Call your provider or go to the emergency room if mental function gets worse.

How do I prevent Frontotemporal Dementia?

There is no known prevention.

Central nervous system and peripheral nervous system
Brain
Brain and nervous system
What are the latest Frontotemporal Dementia Clinical Trials?
Biomarkers in Neurodegenerative Diseases

Summary: With the increase in life expectancy of our population due to advancement of medical diagnosis and treatments, the incidence of age dependent neurodegenerative diseases increased, including Alzheimer's disease (AD), parkinsonian syndromes (PS), small vessel disease (SVD) and motor neuron disease (MND). In spite of the progress of knowing the pathogenesis of various neurodegenerative diseases at mo...

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Investigating if a Stronger tDCS Intensity is More Effective for Improving Naming Ability in People Living With Alzheimer's Disease

Summary: There is currently little symptomatic therapy for Alzheimer's Disease (AD) and nothing effective for individuals with Frontotemporal dementia (FTD). However, neuromodulation with transcranial direct current stimulation (tDCS) has the potential to be a clinically effective therapy for both AD and FTD. The challenge now is to specify the parameters and conditions under which tDCS is most effective t...

What are the Latest Advances for Frontotemporal Dementia?
A Call for Drug Therapies for the Treatment of Social Behavior Disorders in Dementia: Systematic Review of Evidence and State of the Art.
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Effects of Palmitoylethanolamide on Neurodegenerative Diseases: A Review from Rodents to Humans.
Who are the sources who wrote this article ?

Published Date: January 23, 2022
Published By: Joseph V. Campellone, MD, Department of Neurology, Cooper Medical School at Rowan University, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Peterson R, Graff-Radford J. Alzheimer disease and other dementias. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 95.

Paulsen JS, Gehl C. Neuropsychology. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 44.