Bruce Willis’ Diagnosis Casts Rare Spotlight on Frontotemporal Dementia 

DENVER — About a year after actor Bruce Willis announced he would step away from acting, his family announced he’s been diagnosed with frontotemporal dementia. 

Willis was first diagnosed with aphasia, a brain disorder that leads to speaking, reading and writing problems, and is related to frontotemporal dementia, also referred to as frontotemporal degeneration or FTD. 

“FTD is a cruel disease that many of us have never heard of and can strike anyone,” said the statement from Willis’ family. “For people under 60, FTD is the most common form of dementia, and because getting the diagnosis can take years, FTD is likely much more prevalent than we know.”

Initially, reports surfaced that Willis’ aphasia may have been linked to a head injury he sustained in 2002 while doing his own stunts. The connection between head injuries and dementia is not yet well understood for FTD, said Dr. Nicole Purcell, a neurologist and senior director of clinical practice at the Alzheimer’s Association. 

Purcell has not treated Willis, so she can’t say for sure what his case entails, but she said it’s difficult to connect head trauma to FTD. There are no known risk factors for FTD except for family history or a similar disorder, such as aphasia, according to the Alzheimer’s Association. In about a third of all cases, FTD is inherited and there is now genetic counseling and testing available for people with a family history of FTD. 

What Is FTD? 

FTD is a lesser known group of dementia disorders caused by nerve cell loss in the frontal lobes, the region behind the forehead, and the temporal lobes, the region behind the ears. 

There are two main presentations of FTD, Purcell said: the primary progressive aphasia variant and the behavioral variant. People with the aphasia variant have difficulty with speech and language, while the behavioral variant impacts a person’s personality and behavior. 

More research is needed to better understand FTD, but there are two prominent and known diseases that cause the disease — a group of disorders that involve the tau protein and a group of disorders involving the TDP43 protein. 

FTD is far less common than Alzheimer’s disease in those over age 65. Most people with the disease are in the 45-65 age range. It’s estimated there may be between 50,000 and 60,000 people with FTD in the U.S., but it’s likely under-diagnosed, said Purcell. 

How Is FTD Diagnosed? 

“The symptoms that we see with frontotemporal dementia are very insidious or slow in onset,” Purcell said. “If they have the primary progressive form, and they don’t know that initially … they often will notice difficulty with word finding where they’re trying to remember the names of things when they’re speaking. They can’t think of the next word, or they may have difficulty understanding what is being said to them.”

Purcell guessed that Willis probably has the primary progressive aphasia form of FTD, but all she knows is what was released by his family, which didn’t include a distinct form of FTD.

For patients who have the behavioral form, early symptoms can include losing interest in activities they usually enjoy, a loss of social queuing or understanding social norms, and they may have personality changes like becoming more agitated or irritated than normal. 

“Initially patients don’t often pick up on it being a problem, and sometimes they just consider it as part of something that happens with aging,” Purcell said. “They don’t always go to their primary care physician or seek help for these symptoms, at least early on, and then by the time they do present, it’s usually when their symptoms are a little more noticeable.” 

Can FTD Be Treated? 

There are no treatments for FTD, but there are ways to help manage the symptoms. 

“If patients are having problems with speech and language, oftentimes a speech pathologist is very helpful to work with them with regard to their speech or as they start to lose speech [and] other forms of communication,” Purcell said. “If they have more behavioral symptoms, such as agitation or anxiety, we can support them with medications that may be helpful in that regard.”

FTD inevitably gets worse over time, but the speed of disease progression differs from person to person. Patients don’t typically die from FTD, but instead a co-occurring medical condition, Purcell said. 

“As patients progress, they may have difficulty with swallowing. So oftentimes patients will aspirate food or drink causing pneumonia, and it may be the infection that actually causes their death,” Purcell said. “But they can certainly live 10 years and 20 years beyond the diagnosis. It’s just that each individual course is its own individual course.”

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