What is frontotemporal dementia? Actor Bruce Willis, plagued by the disease, is incurable

Actor Bruce Willis’ family announced on Wednesday that he has been diagnosed with frontotemporal dementia, a degenerative brain disease that currently has no cure.

Willis’ wife Emma Heming Willis, ex-wife Demi Moore and daughters Rumer, Scout, Tallulah, Mabel and Evelyn called the diagnosis a “cruel illness” and released their statement on the Assn’s website. for Frontotemporal Degeneration, an advocacy group for patients and their families.

“We know in our hearts that if he could today, he would want to respond by bringing global awareness and a connection with those who are also dealing with this debilitating disease,” they wrote.

An estimated 50,000 people in the US are living with frontotemporal dementia, said Dr. William Seeley, a neurologist at UC San Francisco who received a 2011 MacArthur Foundation “genius” grant for his work on the condition, which he described as a “complex and heterogeneous disease that is difficult to diagnose.”

What is frontotemporal dementia?

Frontotemporal dementia is a progressive brain disorder affecting the anterior and anterior temporal lobes of the brain. It is the most common form of dementia in people diagnosed under the age of 60.

Depending on which part of the brain the disease begins in, the disease initially manifests itself in two ways: with severe changes in behavior or in the ability to speak.

Behavioral variant frontotemporal dementia is diagnosed in just over half of patients. Because the disease destroys cells in key areas of the brain that coordinate social functioning, patients can become suddenly and uncharacteristically uninhibited, saying and doing things that seem inappropriate or impulsive to those around them.

They often appear apathetic or disinterested in activities, said Dr. Mario F. Mendez, neurologist and director of the Behavioral Neurology Program at UCLA. What is most painful for their loved ones is that they can seem distant and unsympathetic to the feelings of those around them.

The remainder of FTD patients fall into the subcategory of primary progressive aphasia, a broad term for speech problems that progressively worsen.

This version of FTD also has two main types. In the non-fluent variety, people may have trouble forming grammatically meaningful sentences — or producing words at all.

Those with the semantic variant can often still speak fluently but lose the ability to understand words.

Willis’ family did not provide details of his frontotemporal diagnosis. They shared his diagnosis of aphasia last year when he announced his retirement from acting. Many who worked with Willis in the last few films he made before he went public with his aphasia diagnosis have noticed changes in the actor’s speech and memory.

“Someone gave him a line and he didn’t understand what it meant,” a crew member for the film “White Elephant” told the Times.

What causes frontotemporal dementia?

The answer is the same as for other types of dementia: we don’t really know.

“For the typical patient, we really don’t have a great way of predicting who is at risk or understanding why they got the disease,” Seeley said.

The disease itself is the result of rogue proteins clumping together in the brain, but what triggers this protein formation is unclear.

Genetics play a role. About 40% of FTD cases are familial in nature, meaning there is a history of dementia in the patient’s family, the Assn. for frontotemporal degeneration.

Most people who are diagnosed with the disease have no family history of dementia at all. At the same time, having a parent or other biological family member with FTD is no guarantee that you will develop the disease.

How is frontotemporal dementia different from Alzheimer’s disease?

“Dementia” is a broad term that encompasses many types of brain damage. The most common and well-known type is Alzheimer’s disease, which typically begins in the hippocampus, a part of the brain essential for memory formation. That is why forgetting is the first and most characteristic symptom of the disease.

The disease then usually progresses through the brain in a predictable pattern, triggering new symptoms as it atrophies tissue in different regions. From the hippocampus, it travels further to the amygdala and provokes feelings of fear and anxiety; to the parietal lobes, disturbing bodily sensation and spatial awareness; and finally to the frontal lobes, which manage memory retrieval, problem solving, sensory processing, and communication.

But for people with frontotemporal dementia, the damage starts there first. While personality changes, loss of inhibition, and difficulty remembering and maintaining relationships occur in the late stages of Alzheimer’s, people with FTD face these extremely difficult symptoms much earlier in the disease process.

“Whether it’s a behavioral or a verbal form of the disease, it takes away aspects of personality that are really central to us. And that’s why it’s so hard for the patients and the families,” Seeley said.

What is the prognosis and treatment?

The number of years people with frontotemporal dementia can live varies greatly from patient to patient, Mendez said.

The Assn. for frontotemporal degeneration states that the average life expectancy of patients is between seven and 13 years once symptoms appear.

Some medications can help relieve some of the symptoms of the disease. But at the moment, no treatment can stop or reverse the disease itself.

“Unfortunately,” Mendez said, “a key aspect of neurodegeneration is that it is insidious and progressive. And so it eventually leads to death, as many of these diseases do.”

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