What you need to know about frontotemporal dementia

Brain scan

Bruce Willis whose condition has progressed from aphasia to frontotemporal dementia, is suffering from “a cruel disease that many of us have never heard of and can strike anyone,” the actor’s family said in a statement.

Since his diagnosis, talk show host Wendy Williams also has been diagnosed with the same condition.

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Frontotemporal dementia is a condition that can change a person’s behavior and hampers their communication skills. So, what is this complicated disease?

According to Johns Hopkins Medicine, frontotemporal dementia -- also known as FTD -- is a group of disorders that occur when nerve cells in the frontal and temporal lobes of the brain are lost. This causes the lobes to shrink.

On its website, the Mayo Clinic said that some people with FTD experience dramatic changes to their personalities and become socially inappropriate, impulsive, or emotionally indifferent. Other people lose the ability to use language properly.

“It hits the parts of the brain that make us the most human,” Dr. Bruce Miller, a professor of neurology at the University of California, San Francisco, told The New York Times.

There are two types of FTD, according to the newspaper. One is primary progressive aphasia, which hampers a patient’s ability to communicate, while the other is behavioral variant frontotemporal dementia, in which a person exhibits personality and behavioral changes.

There is a less common variant of FTD, according to Johns Hopkins Medicine. It affects movement and causes symptoms similar to those experienced by people diagnosed with Parkinson’s disease or amyotrophic lateral sclerosis, which is commonly known as Lou Gehrig’s disease.

According to the Association for Frontotemporal Degeneration, FTD commonly affects people younger than 60, although there are cases where people much older can be affected.

There are no approved treatments for FTD, and there is no cure, the association said on its website. People diagnosed with FTD face a life expectancy between seven and 13 years.

“Just like Alzheimer’s, at this time, there is no disease-modifying therapy, no curative therapy,” Chi-Ying “Roy” Lin, a neurology professor at Baylor College of Medicine, told The Washington Post.

Approximately 50,000 people in the U.S. have been diagnosed with the condition, Susan Dickinson, the chief executive of the Association for Frontotemporal Degeneration, told the Times.

However, Dickinson told the newspaper that the figure could be higher because the condition can be challenging to diagnose.

People suffering from the behavioral variant of FTD “seem like they’ve lost a bit of their filter,” Dr. Ian Grant, an assistant professor of neurology at the Northwestern University Feinberg School of Medicine, told the Times. Some people may also display a lack of empathy for people around them, Grant told the newspaper.

Patients with the behavioral variant rarely have memory issues, CNN reported. However, they do have organizational issues and have trouble setting their priorities, according to the National Institute on Aging.

In primary progressive aphasia, a person may have trouble speaking or understanding words and might even slur their speech, CNN reported.

“PPA may start with difficulty simply finding words, so people begin to use simpler words or more generic words for things they can’t quite recall,” Paulson told the cable news network. “Now that also comes with the territory of aging, but when the language is more effortful on a daily basis, or the comprehension is going downhill, that’s a sign that someone should see a doctor for an evaluation.”

While FTD may appear to mirror Alzheimer’s disease, there are some differences.

Memory loss is more prominent in early Alzheimer’s patients than with people affected by FTD, according to the Alzheimer’s Association. People who have problems with spatial orientation -- like getting lost in familiar places -- are also more common to Alzheimer’s patients.

Hallucinations are more common for Alzheimer’s patients than for those with FTD, the website noted.

Are there hereditary risks of being diagnosed with FTD? According to John Hopkins Medicine, a family history of FTD is the only known risk. However, most people with FTD have no family history of that condition or other types of dementia.

Frontotemporal dementia used to be called Pick’s disease, according to the Alzheimer’s Association. It was named for Arnold Pick, a physician who in 1892 first described a patient with distinct symptoms affecting language. Some doctors still use the term “Pick’s disease.”

Even though the disease can progress, patients can continue to lead an active lifestyle, Paulson said.

“I’ve seen patients who completely lose their speech and yet they go out and take their camera and take beautiful photographs of the lives they’re living,” Paulson told CNN. “They can’t tell me in words, but they can tell me in pictures.

“I tell all my patients, ‘Don’t let this disease own you. You own it.’ Sure, you’ve lost some skills because of the illness you have, but you still have lots of skills left and you work with the skills you have.”

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