Frontotemporal Dementia (FTD)

Pick’s Disease
Pick’s Disease

Overview Of Frontotemporal Dementia (FTD)

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

Commonly Associated With

Semantic Dementia; Dementia – semantic; Frontotemporal dementia; FTD; Arnold Pick disease; Pick disease; 3R tauopathy

Causes Of Frontotemporal Dementia (FTD)

People with FTD have abnormal substances (called tangles, Pick bodies, and 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.

Symptoms Of Frontotemporal Dementia (FTD)

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’s disease. (Memory loss is often the main, and earliest, symptom of Alzheimer’s 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).

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 the 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

Exams & Tests

The health care provider will ask about the medical history and symptoms.

Tests may be ordered to help rule out other causes of dementia, including dementia due to metabolic causes.

FTD is diagnosed based on symptoms and results of tests, including:

  • Assessment of the mind and behavior (neuropsychological assessment)
  • Brain MRI
  • Electroencephalogram (EEG)
  • Examination of the brain and nervous system (neurological exam)
  • Examination of the fluid around the central nervous system (cerebrospinal fluid) after a lumbar puncture
  • Head CT scan
  • Tests of sensation, thinking, and reasoning (cognitive function), and motor function
  • Newer methods that test brain metabolism or protein deposits may better allow for more accurate diagnosis in the future
  • Positron emission tomography (PET) scan of brain
  • A brain biopsy is the only test that can confirm the diagnosis.

Treatment Of Frontotemporal Dementia (FTD)

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:

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 families 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.