Frontotemporal dementia (frontotemporal lobar degeneration) is an umbrella term for a diverse group of uncommon disorders that primarily affect the frontal and temporal lobes of the brain — the areas generally associated with personality, behavior and language.
In frontotemporal dementia, portions of these lobes atrophy or shrink. Signs and symptoms vary, depending upon the portion of the brain affected. Some people with frontotemporal dementia undergo dramatic changes in their personality and become socially inappropriate, impulsive or emotionally indifferent, while others lose the ability to use and understand language.
Frontotemporal dementia is often misdiagnosed as a psychiatric problem or as Alzheimer's disease. But frontotemporal dementia tends to occur at a younger age than does Alzheimer's disease, typically between the ages of 40 and 70.
Identifying precisely which diseases fall into the category of frontotemporal dementia presents a particular challenge to scientists. The signs and symptoms may vary greatly from one individual to the next. Researchers have identified several clusters of symptoms that tend to occur together and are dominant in subgroups of people with the disorder. More than one symptom cluster may be apparent in the same person. The signs and symptoms of frontotemporal dementia progressively worsen with time, almost always over years, eventually requiring 24-hour care.
Speech and language problems
Movement-related signs and symptoms may include:
A variety of mutations on several different genes have been linked to specific subtypes of frontotemporal dementia. But more than half the people who develop frontotemporal dementia have no family history of dementia.
In some cases of frontotemporal dementia, the affected parts of the brain contain microscopic Pick bodies — abnormal protein-filled structures that develop within brain cells. Frontotemporal dementia was once known as Pick's disease, but now that terminology is reserved for the subtype that actually features these abnormal structures.
Your risk of developing frontotemporal dementia is higher if you have a family history of dementia. There are no other known risk factors.
Preparing for your appointment
People who have frontotemporal dementia often don't recognize that they have a problem. In many cases, family members are the ones who notice the symptoms and arrange for a doctor's appointment. Your family physician may refer you to a neurologist or psychologist for further evaluation.
What you can do
What to expect from your doctor
During the appointment, your doctor might also conduct a brief mental status evaluation, which may assess:
Tests and diagnosis
No single test can identify frontotemporal dementia, so doctors attempt to identify certain characteristic features while excluding other possible causes.
The image on the right, particularly the area near the white arrow, shows the brain shrinkage common in frontotemporal dementia. ...
Treatments and drugs
There's no cure for frontotemporal dementia and no effective way to slow its progression. Treatment relies on managing the symptoms.
Lifestyle and home remedies
In some cases, caregivers can reduce behavior problems by changing the way they interact with people who have dementia. Examples include:
Coping and support
If you've been diagnosed with frontotemporal dementia, receiving support, care and compassion from people you trust can be invaluable. Through your doctor or the Internet, find a support group for people with frontotemporal dementia. You'll find information tailored for your needs, as well as a forum that gives you the opportunity to share your experiences and feelings.
When a person with frontotemporal dementia requires 24-hour care, most families turn to nursing homes. Plans made ahead of time will make this transition easier and may allow the person to be involved in the decision-making process.
Last Updated: 2011-10-19
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