By Kory Grow
Bruce Willis’ family announced on February 16 that the actor has been diagnosed with frontotemporal dementia (FTD) – a rare condition that will severely impact his quality of life. According to the Association for Frontotemporal Degeneration, FTD is a group of disorders associated with atrophy of the frontal and/or temporal lobes of the brain. In a joint statement, Willis’s family described FTD as a “cruel disease”.
dr Joel Salinas, an assistant professor of neurology at New York University Langone Health, is a behavioral neurologist who has researched FTD and Alzheimer’s. He explained to ROLLING STONE how the disease affects those affected, how it can be recognized and treated. His research indicates that FTD is extremely difficult to live with, Salinas said. The question of the quality of life of a person with this diagnosis is “heartbreaking”.
“As the disease progresses, the quality of life is impacted quite severely,” he says. “There was a study that looked at how loved ones’ relatives rated their loved one’s condition and the most common response was ‘worse than dead.'”
FTD develops when a person accumulates misfolded proteins in the frontal and/or temporal lobes of the brain. The accumulation of these proteins over time leads to inflammation and damage to the nerve cells. Symptoms depend on which part of the brain is affected by the disease.
forms of the disease
Behavioral symptoms are common when regression occurs in the frontal lobes. “This can manifest itself as slow behavioral changes that include personality changes, disinhibition, impulsivity, new obsessions and compulsions,” explains the neurologist. “Frequent reports include broken relationships with family members and friends, divorce, poor job performance, gambling, overspending, and sexual leaks. This upsets family members and causes significant social disruption.”
In particular, people who suffer from FTD in the temporal lobes often have problems with language and communication. “This is where the so-called primary progressive aphasia develops, as occurs in Bruce Willis,” says Salinas. “It manifests itself in difficulties in finding words, in understanding, or in writing. Over time, there may even be difficulty forming the words with the mouth. As the disease progresses, people may have difficulty swallowing, which puts them at greater risk of pneumonia.”
A third group, less common, relates to muscle function. It can look like Parkinson’s disease – sufferers complain of muscle weakness. According to Salinas, people with a family history of ALS (Lou Gehrig’s disease) are more susceptible to this form of FTD. “It’s important to realize that about 20 to 30 percent of people who develop FTP have a familial or genetic cause,” he says. “A lot of research needs to be done to really find out what causes the rest of the people.”
The diagnosis
The lack of research results explains why many patients are often diagnosed late – three years after the onset of the disease. Patients are often already suffering from mild behavioral problems when doctors diagnose the disease through clinical tests. FTD becomes “dementia” when a person is unable to perform everyday activities. The degree of the disease can be mild, moderate or severe. The way the disease progresses varies from person to person and can last anywhere from seven to 13 years. However, Salinas is aware of cases where the disease has progressed in as little as two years or in 20 years. People can develop FTD from the age of 20 through their 80s. Most diagnoses are made between the ages of 45 and 65.
“Mild impairments concern everyday things like dealing with correspondence and finances, public transport, the ability to get up and go to work,” explains the professor. “Once someone gets into the middle stage, we see more dependency on the basic activities of daily living: showering, dressing, using the toilet, and difficulty eating. If someone is then very dependent in almost all areas, one speaks of a severe form. When the disease is so advanced that the patient not only has difficulty swallowing but also has difficulty eating and has very limited ability to interact, we call this ‘end stage’.”
treatment options
In their statement, the Willis family said there are no treatments for the disease. Salinas says doctors work with patients based on their symptoms. They would put a patient in touch with speech and cognitive therapists to help their communication, and in some cases, if necessary, prescribe medication such as antipsychotics for people in severe distress. But: “There is no cure for this disease. Ultimately, doctors are still learning about this disease.”
The lack of research results is a consequence of the lack of awareness of this disease. “Not many people know about it, even though it’s the most common form of dementia in people over 60,” he says. “And those affected are often unable to stand up for themselves.” In particular, the neurologist would like more research into the biological mechanism of the disease. Also, more clinical trials should be conducted to determine whether experimental drugs are effective in the early stages of the disease.
Salinas hopes that early symptoms, such as B. word-finding disorders, are better recognized and the disease can be diagnosed earlier. If people can better understand FTD and doctors can do more research, it could greatly improve the lives of those affected. “The costs can sometimes be double what it is for people with Alzheimer’s,” he says.
Willis’ family announced in March 2022 that the actor was retiring from the film business due to aphasia. “As Bruce’s condition continues to progress,” the family wrote in their statement, “we hope media attention can focus on shedding light on this disease, which needs much more awareness and research.”
Translated from the American, first published on rollingstone.com
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