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What is frontotemporal dementia?

Susan Barber Lindquist, Mayo Clinic News Network on

Published in Health & Fitness

"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 primary progressive aphasia subtypes of FTD worldwide is uncertain. Estimates are between 15 to 22 per 100,000 people who are 45 to 64 years old have FTD. In the U.S., behavioral variant frontotemporal dementia and primary progressive aphasia are estimated to affect 50,000-60,000 people.

Other disorders also are grouped under the FTD umbrella:

• Amyotrophic lateral sclerosis, also called Lou Gehrig's disease, causes lack of muscle control. Muscle twitching and limb weakness or slurred speech often are the first signs.

• Corticobasal syndrome results in poor coordination and stiffness in the arms or legs (often worse on one side of the body than the other), difficulty thinking, and trouble with speech and language.

• Progressive supranuclear palsy causes problems with walking, balance, eye movements and swallowing.

 

If FTD disorders have so many similarities, how are they diagnosed?

Determining a diagnosis can be challenging. Patients with behavioral variant frontotemporal dementia sometimes are misdiagnosed with a psychiatric or stress-related illness, such as atypical depression, late-onset bipolar disorder or schizophrenia, or even midlife crisis, Dr. Boeve says.

With assistance from tests to help rule out other disorders, a doctor diagnoses a patient based on symptoms. For example, if patients are having more behavior and personality changes — with or without any language or motor problems — if they have trouble with problem-solving and judgment, but memory is relatively preserved, that's more typical of behavioral variant frontotemporal dementia, Dr. Boeve says.

Brain imaging tests are important. An MRI scan may show shrinking of the frontal or temporal lobes of the brain. A PET scan may show metabolic changes in these same regions of the brain.

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