Bruce Willis has Frontotemporal Dementia: What is FTD and Its Symptoms?

REUTERS/Henry Nicholls/File Photo
Actor Bruce Willis attends the European premiere of “Glass” in London, Britain January 9, 2019.

Actor Bruce Willis has been diagnosed with frontotemporal dementia (FTD), a rare type of dementia, his family announced Thursday. The disease, also known as frontotemporal lobar degeneration, has no treatment or cure.

Willis’s family said in March that he had been diagnosed with aphasia, a communication disorder, and was retiring. In their announcement Thursday, Willis’s family said his “condition has progressed and we now have a more specific diagnosis: frontotemporal dementia.”

“While this is painful, it is a relief to finally have a clear diagnosis,” said their statement posted on the website for the Association for Frontotemporal Degeneration. “FTD is a cruel disease that many of us have never heard of and can strike anyone.”

We asked neurologists questions about the condition, its causes, symptoms, impact and risks, and here are their answers.

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What is frontotemporal dementia?

Frontotemporal dementia is a category of neurodegenerative diseases in the same family as Alzheimer’s disease and Parkinson’s. About 50,000 to 60,000 people in the United States have FTD. It mostly affects people between the ages of 40 and 60.

The most accurate term would be frontotemporal lobar degeneration, which is more specific to the process going on in the brain, said Joel Salinas, a behavioral neurologist, assistant professor of neurology at NYU Langone Health and chief medical officer of Isaac Health.

FTD is a category of dementias that specifically affects the brain’s frontal and temporal lobes. “Sometimes it’s more the frontal, sometimes it’s more the temporal, and over time it usually involves both,” Salinas said.

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What causes frontotemporal dementia?

Abnormal proteins such as tau and TDP-43 accumulating in the brain is the most likely cause of FTD, the experts said. In the long term, these misfolded disease proteins cause neurotoxicity and, ultimately, cell death.

There are at least 15 gene mutations associated with FTD, and each may be a “different type of FTD,” said Paul Schulz, professor of neurology and neurodegenerative disorders at the McGovern Medical School of UTHealth Houston. “That means that each could potentially have different causes.”

There also are many patients with FTD without an identified genetic mutation. Their FTD may have an environmental cause such as head trauma, though more work is needed to confirm this, Schulz said.

“It’s a relatively recently described disease,” said Constantine Lyketsos, director of the Johns Hopkins Memory and Alzheimer’s Treatment Center and professor of psychiatry and behavioral sciences. “We’ve heard of Alzheimer’s for over 100 years” but FTD is much less common, he said. “So the fact that it’s more recent and less common means we know much less about it than we’d like to.”

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What are the symptoms of frontotemporal dementia?

Symptoms can vary and depend on where the abnormal proteins begin to accumulate – in the frontal or temporal lobe. It can take a few years for a patient to be diagnosed with FTD since the symptoms are varied and also may be seen in people with other diseases, the physicians said.

A patient with frontal lobe-focused abnormality would show behavioral issues of impulsivity and disinhibition. That’s called behavioral variant activity, which is the more common subvariant of FTD.

“For example, a polite person may become rude and a kind person may become self-centered,” said Andrew Budson, chief of cognitive and behavioral neurology, associate chief of staff for education, and director of the Center for Translational Cognitive Neuroscience at the Veterans Affairs Boston Healthcare System. “There may also be a lack of self-control that sometimes causes overeating of foods, such as an entire jar of mayonnaise, which one of my patients ate.”

Social disinhibition is one symptom, said Ryan Darby, assistant professor of neurology and director of the Frontotemporal Dementia Clinic at Vanderbilt University Medical Center. “They may even commit crimes because of their disinhibition and socially inappropriate behaviors,” he said. “They lose empathy and compassion toward others.”

When the abnormality develops in the temporal lobe, in particular the temporal side of the brain that involves speech and language, a patient could have primary progressive aphasia, which is what Willis has. People with aphasia find it difficult to understand language or produce language.

There are two major subtypes of aphasia – the agrammatic type or the semantic type – that are caused by FTD, said Brad Dickerson, director of the Frontotemporal Disorders Unit at Massachusetts General Hospital, neurologist and professor of neurology at Harvard Medical School. “The agrammatic type is more of a problem with producing grammatically constructed sentences, syntax, whereas the semantic type is really the meanings of words,” he said. “The agrammatic type tends to be due to the tau problem in the brain, which affects the frontal lobe, mostly. Whereas the semantic type tends to be due to the TDP-43 type, which tends to affect the temporal lobe of the brain.”

Some others can develop abnormal protein in parts of the brain associated with motor functioning and eye movement, the experts said.

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What are the signs or stages of frontotemporal dementia?

The first signs of FTD can be subtle, such as alterations in behavior, speech or movement, the experts said.

Neurodegenerative diseases such as FTD begin with small changes, what are known as subjective cognitive or behavioral concerns or decline, that often are not detected in clinical testing, Salinas said.

When these changes progress and can be detected, they are described as mild cognitive impairment or mild behavioral impairment.

When these impairments begin to interfere with day-to-day activities, they can be defined as dementia. Dementia has at least three stages – mild, moderate and severe.

Patients with end stage dementia show little interactivity and have difficulty eating and swallowing.

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How fast does frontotemporal dementia progress?

Progress of the disease can vary, the neurologists said.

The average life expectancy is about 7 to 13 years; however, some patients die within a few years, and others can live 20 years after diagnosis, Salinas said.

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Can frontotemporal dementia be treated?

There is no cure for FTD. “Just like Alzheimer’s, at this time, there is no disease-modifying therapy, no curative therapy,” said Chi-Ying “Roy” Lin, a neurology professor at Baylor College of Medicine.

The effectiveness of pills that affect memory or cognition were based on the autopsy findings of Alzheimer’s, Lin said. These cholinesterase inhibitors, known as Donepezil or Rivastigmine, might be used in the mild stages of Alzheimer’s and FTD. The second treatment is memantine, an NMDA receptor antagonist. “And typically, we would add that in the moderate to severe stage,” he said.

“In people with Alzheimer’s, by adding this medication, the cognitive decline rate could be potentially slower,” Lin said. “However, in FTD patients, there’s no such convincing data. But that’s just what we have.”

Physicians focus on helping patients and their families manage symptoms of the disease. Patients may need help managing issues such as anxiety, depression, aggressiveness, agitation and obsessional behavior. The SSRI (selective serotonin reuptake inhibitors) medications in the Prozac family of drugs have been shown to help with some of the problems in behavioral variant frontotemporal dementia, said Budson, who also is a professor of neurology at Boston University.

Speech therapy has been shown to be helpful for initial stages of primary progressive aphasia, the experts said, but no medications have been shown to be effective.

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Who is at risk of frontotemporal dementia?

Frontotemporal dementia, as a category of dementias, can affect anyone, though some cases have a genetic predisposition.

“There’s a family history, some type of dementia or neurodegenerative disease in about 40 percent of cases,” Darby said. “And in about 10 percent of cases, there’s an actual gene that can be identified that’s autosomal dominant. So it means that if you have the gene, then you’re likely or almost certainly will develop the disease at some point.”

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How does FTD compare with other types of dementia?

FTD differs from other types of dementia in the typical age of onset. FTD is most common among people under 60.

In FTD, specific areas of the brain – the frontal and temporal lobes – are affected, affecting behaviors, speech and some motor skills. In disorders such as Alzheimer’s disease, the degeneration is seen more in the temporal lobes, especially the hippocampus, as well as the parietal lobes, Salinas said. Memory loss is often the first symptom of Alzheimer’s disease.

Unlike Alzheimer’s disease, the risk of frontotemporal dementia does not increase with age, the experts said.

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What kind of care would someone with FTD need?

The kind of care would depend on the stage of the disease, the experts said. Patients may need help with physical safety and communication, for instance. Behavioral issues may need redirection.

“For example, if judgment changes early, then driving and taking care of one’s own finances can be dangerous,” Schulz said. Being gullible due to lacking insight, “they may donate their money to nefarious persons, or may marry someone who might be looking to inherit their savings.”

And as the disease progresses, patients may need greater support with day-to-day activities, including over time with getting dressed, using the toilet, showering and eating.

“There’s a worldwide effort to try to fight back against these diseases and make progress toward their earlier diagnosis and toward their better treatment,” said Dickerson, who also is the Chair of the National Medical Advisory Committee of the Association for FTD. “And a major part of that is raising awareness. It is critically important for someone like Bruce Willis and his family to speak up about this, because there’s so much stigma associated with any type of dementia still.”

It makes others living with this condition feel less alone, he said.