The family of Bruce Willis announced that the actor has frontotemporal dementia, known as FTD, a form of dementia that occurs most commonly when nerve cells in the frontal and temporal lobes of the brain decrease in number. Mr. Willis, 67, was previously diagnosed with aphasia, which prompted him to retire from acting. “FTD is a cruel disease that many of us have never heard of and can strike anyone,” the family wrote in a statement.
There are two main variants of FTD: primary progressive aphasia, which hampers a patient’s ability to communicate, and behavioral variant frontotemporal dementia, which manifests as personality and behavioral changes. “It hits the parts of the brain that make us the most human,” said Dr. Bruce Miller, a professor of neurology at the University of California, San Francisco.
FTD is the most common cause of dementia for people under the age of 60, said Susan Dickinson, the chief executive of the Association for Frontotemporal Degeneration. There are roughly 50,000 people in the United States with a diagnosis of FTD, she added, although many experts consider that number to be a vast undercount, because of how challenging it can be to diagnose. There is no blood test or single biomarker to diagnose the condition — doctors instead identify it based on symptoms and neuroimaging. On average, it takes patients more than three years to get an accurate diagnosis, Ms. Dickinson said.
What are the first signs of frontotemporal dementia?
People with primary progressive aphasia may struggle to speak in full sentences or have difficulty comprehending conversations. They may have a hard time writing or reading.
Those with the behavioral variant of FTD may act out of character, said Dr. Ian Grant, an assistant professor of neurology at the Northwestern University Feinberg School of Medicine. Families will say that patients “seem like they’ve lost a little bit of their filter,” he said. Someone who is typically quiet and reserved may start spewing profanities, for example, or loudly comment on a stranger’s appearance. The person may act apathetic, Dr. Miller said, losing motivation. Some may also display a lack of empathy for those around them.
People with FTD often struggle with organizational skills, failing to multitask or plan. Frontotemporal dementia can also manifest in excessive gambling or impulsive spending, said Dr. Joel Salinas, a clinical assistant professor of neurology at NYU Langone Health.
Dr. Grant described the poor judgment and erratic behavior that can come with personality changes from FTD, such as drinking an entire bottle of maple syrup. “They may be craving instant gratification, without any real ability to put a check on that,” he said.
In some cases, patients may be misdiagnosed with a psychiatric condition, like bipolar disorder, because of these symptoms, Dr. Grant added. There are a few ways to distinguish between a psychiatric condition and FTD, though: Frontotemporal dementia typically affects people in their 50s and 60s, and it is unusual, although not impossible, he said, for people to develop a new psychiatric disorder at that age without a previous history of mental health concerns.
Also, a brain scan in a person with FTD can show atrophy or shrinking in the front of the brain, while a psychiatric illness would be less likely to show such changes.
What causes frontotemporal dementia?
Genetics are a critical risk factor for FTD, but beyond that, doctors aren’t sure what causes the disease in most people, Dr. Miller said. There are, however, things that even people with a family history of the illness can do to lower their risk of developing dementia or to slow the disease course, he said, including exercising, engaging with others and keeping up with cognitive activities like reading, crossword puzzles, learning a new language or playing a musical instrument. But there isn’t evidence that any one activity can prevent FTD, Ms. Dickinson said.
What are the treatment options for FTD?
Generally, patients with FTD will live for around five to seven years after they first display obvious symptoms, Dr. Grant said. There is no cure — “but there’s a difference between a disease not having a cure and saying, ‘There’s nothing we can do,’” he said.
Occupational therapy can help patients who have difficulty communicating. Speech therapy can also be a tool to help these patients, Dr. Salinas said.
Physicians often work with the families or loved ones of people with FTD to develop a care plan, which can include safeguards to make sure they cannot hurt themselves or their loved ones. Doctors may prescribe medications to help manage the symptoms; some patients may be prescribed antidepressants to cope with mood or behavioral symptoms, Dr. Miller said.
Patients often benefit from steady, predictable routines, Ms. Dickinson said. And many patients, as well as family members, find comfort in support groups, she added.
Doctors also work to ensure that families are looking out for themselves. “The family often suffers as much as the patient,” Dr. Miller said.
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