Bruce Willis‘ Frontotemporal Dementia Diagnosis Explained: A ’Heartbreaking’ Disease

Bruce Willis’ family announced today that the actor has been diagnosed with frontotemporal dementia (FTD), a rare disease that will greatly affect his quality of life. Loosely defined, FTD represents a group of disorders that accompany degeneration of the frontal and/or temporal lobes of the brain, according to the Association for Frontotemporal Degeneration, which the Willis family has referred to. In a joint statement, Willis’ family described FTD as a “cruel disease.”

Dr. Joel Salinas, who is Assistant Professor of Neurology at NYU Langone Health and Chief Medical Officer at Isaac Health, is a behavioral neurologist who has studied FTD and Alzheimer’s. He spoke with Rolling Stone to offer clarity on how FTD affects those who are diagnosed with the disease and how it can be both detected and treated. He says research he has reviewed suggests the disease is exceedingly difficult to live with. When asked to describe the quality of life of someone diagnosed with FTD, he calls it a “heartbreaking question.”

“As the condition progresses, quality of life is pretty significantly affected,” Dr. Salinas says. “There was one study that actually looked at caregivers’ [perspective on] the quality of life of their loved ones with FTD, and particularly behavioral variant FTD, and the more common kind of response was ‘worse than dead.’”

FTD develops when a person accumulates abnormal proteins in the brain’s frontal and/or temporal lobes. As the proteins build, the nerve cells suffer inflammation and injury over time. Symptoms depend on which part of the brain the disease is affecting.

Degeneration in the frontal lobes tend to develop behavioral symptoms. “This can present with slow changes in behavior that includes personality changes, disinhibition, impulsivity, new obsessions, and compulsions,” he says. “Oftentimes, the types of things that are reported are fractured relationships with family members and friends, divorce, poor job performance, gambling, excessive spending, sexual indiscretions, and saying and doing things that can make people really upset or embarrassed with pretty notable social impairment.”

People suffering from FTD in the temporal lobes suffer most with speech and communication. “That’s where people develop what’s called primary progressive aphasia, which is the case for Bruce Willis, which is an unfortunate situation,” Dr. Salinas says. “It presents as difficulty with word finding, comprehension, or writing. Over time, it can even evolve into difficulty with producing words with the mouth itself. As the disease progresses, particularly the temporal lobe, people can start to have difficulty with things like swallowing, which places them at a higher risk of developing pneumonia.”

A third group, which is less common, affects motor issues and muscle function. It can appear like Parkinson’s, as people report weakness with muscles. Dr. Salinas says that people who have ALS (Lou Gehrig’s Disease) in their family, are more prone to this form of FTD. “It is important to note that for about 20 to 30 percent of people who develop FTP, it’s because of a familial or genetic cause,” he says. “There’s a lot of research still that needs to happen to really better figure out what causes these conditions for the rest of the people.”

The dearth of available research explains why diagnosis often comes late for many patients, as late as three years into developing the disease. Often a patient is experiencing mild cognitive or behavioral impairment by the time doctors detect the disease through clinical testing. FTD becomes “dementia,” when a person can no longer carry out day-to-day activities. The degree to which someone has FTD can be mild, moderate, or severe. The way the disease progresses varies by person and can take place over seven to 13 years, though Dr. Salinas is aware of cases that have progressed as rapidly as over two years and as slowly as over 20. People can develop FTD in their 20s and as late as their 80s, though most receive FTD diagnoses between ages 45 and 65.

“Mild impairment of affects day to day things like managing correspondence and finances, public transport, being able to get up have a job,” Dr. Salinas says. “Once somebody advances into the moderate stage, we see more dependence in the basic activities of daily living: showering, dressing, toileting, and difficulty with eating. Then once somebody’s very dependent on just about everything, that would be severe. When somebody is so progressed to where they are having difficulty with not just swallowing but also with eating and having very limited interaction, we call that ‘end stage.’”

In their family statement, the Willises said there were no treatments for the disease, though Dr. Salinas says doctors work with patients depending on their symptoms. They would connect a patient with speech and cognitive therapists to help a patient’s communication and, in some cases, prescribe medications like antipsychotics for people in severe distress if necessary. But Dr. Salinas affirms, “There are no cures for the condition.” Ultimately, doctors are still learning about the disease.

The lack of available research is a result of a lack of awareness for the disease. “Not many people know about it, despite it being the most common form of dementia under age 60,” Dr. Salinas says. “And people who are affected by it aren’t often able to advocate for themselves, so you don’t see as many people speaking to Congress, for example, to demand more research.” Specifically, Dr. Salinas would like to see more research on the biological mechanism of the disease: why the proteins develop in the frontal lobes to begin with. There also need to be more clinical trials to decide whether experimental medications work when prescribed earlier in the diagnosis process. “Once we begin to lose those brain cells, it might be too late,” he says.

Another issue that patients diagnosed with FTD face is a lack of specialists to treat them. “There aren’t very many behavioral neurologists in the country,” Dr. Salinas says. “There are some estimates of about like, 370 behavioral neurologists. There was one study out of the Society of Actuaries looking at kind of workforce issues, and they found that half the country was essentially defined as a dementia, neurology desert where there are 10 or fewer specialists in these areas for every 10,000 people who have any form of dementia, let alone people who are beginning to experience the early symptoms of these conditions.”

What Dr. Salinas hopes for is more awareness of the early symptoms, such as having trouble finding words, which could lead to earlier diagnosis. As people understand the disease more and doctors are able to do more research, it could greatly improve the lives of those affected. “The cost can be sometimes twice as high compared to people with Alzheimer’s disease,” Dr. Salinas says.

Willis’ family announced in March 2022 that the actor would be retiring from film as he dealt with what was then believed to be aphasia. “As Bruce’s condition advances,” the family wrote in its statement today, “we hope that any media attention can be focused on shining a light on this disease that needs far more awareness and research.”

From Rolling Stone US.