How Bruce Willis' form of dementia affects sufferers

It was almost a year ago when the show business world and millions of fans worldwide learned the heartbreaking news about movie star Bruce Willis: that the Hollywood icon been diagnosed with aphasia — a neurological disorder that affects the sufferer’s ability to understand and/or produce language.

The diagnosis meant that Willis, 67, would be forced to step away from acting.

Last month, via a statement released by Willis’ family, came more unfortunate news about his health.

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On Instagram, Willis’ wife Emma Heming Willis, former wife Demi Moore and his three adult daughters (Rumer, Scout and Tallulah) with Moore posted the following in a joint statement: “We wanted to give you an update about our beloved husband, father and friend since we now have a deeper understanding of what he is experiencing. Since we announced Bruce’s diagnosis of aphasia in spring 2022, Bruce’s condition has progressed and we now have a more specific diagnosis: frontotemporal dementia (known as FTD). Unfortunately, challenges with communication are just one symptom of the disease Bruce faces. While this is painful, it is a relief to finally have a clear diagnosis.”

In a more comprehensive joint statement posted to The Association for Frontotemporal Deterioration website (theaftd.org), the women noted “FTD is a cruel disease that many of us have never heard of and can strike anyone ...[and] as Bruce’s condition advances, we hope that any media attention can be focused on shining a light on this disease that needs far more awareness and research.”

In the week after the news broke, People magazine reported that the family’s “focus for Bruce is to keep him active. He has a busy schedule with activities every day. They make sure both his body and brain [are] exercised.”

Dementia isn't just Alzheimer's

The National Institute on Aging says there are three types of frontotemporal dementia: behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and movement disorders.

It’s helpful to understand that the word “dementia” is an umbrella term for several neurological conditions in which the primary symptoms include a significant decline in brain function.

For instance, Alzheimer's disease is the most common type of dementia and generally affects the temporal and parietal lobes of the brain. It’s progressive and invariably fatal.

Vascular dementia is caused by damage to the vessels that supply blood to the brain.

Lewy body dementia happens when abnormal structures, called Lewy bodies, build up in areas of the brain. The disease may cause a wide range of symptoms, including changes in alertness and attention, hallucinations, problems with movement and posture, muscle stiffness, and confusion.

Lewy body dementia is the disease that actor and comedian Robin Williams was suffering from when he died by suicide in 2014.

According to Cleveland Clinic Weston neurologist and dementia specialist Dr. Damon Salzman “frontotemporal dementia primarily affects the frontal and temporal lobes of the brain — the areas generally associated with personality and behavior, as well as memory and language.”

The condition, says the Association for Frontotemporal Degeneration, typically strikes sufferers between the ages of 45 and 64 and is the most common form of dementia for people younger than 60.

A CNN report noted that “frontotemporal dementia is a group of disorders caused by a buildup of tau and other brain cell-destroying proteins.”

The fact that Willis’ condition started with difficulty speaking means he has primary progressive aphasia.

Salzman noted that “most cases of aphasia actually don’t progress to FTD because the aphasia usually occurs as a result of stroke, brain tumor, brain lesion or traumatic brain injury.”

Aphasia is typically result of stroke, tumor or trauma — but not when it is signal of frontotemporal dementia (FTD)

There are three types of primary progressive aphasia FTD, and they’re differentiated by the kind of language problems that first appear:

• Semantic PPA: A person slowly loses the ability to understand single words and sometimes to recognize the faces of familiar people and common objects.

• Agrammatic PPA: A person has increasingly more difficulty speaking and may omit words that link nouns and verbs. Eventually, the person may no longer be able to speak at all.

• Logopenic PPA: A person has trouble finding the right words during a conversation but can understand words and sentences.

FTD often mistaken for mental health issue

FTD can be hard to diagnose at first because the symptoms are similar to those of other conditions.

Oftentimes, the condition is misdiagnosed as a mood disorder, such as depression.

And as the National Institute on Aging says “to make matters more confusing, a person can have both FTD and another type of dementia, such as Alzheimer's disease. Also, because these disorders are rare, physicians may be unfamiliar with the signs and symptoms.”

In most cases, says the National Institute on Aging, “the cause of a FTD is unknown. Individuals with a family history of FTD are more likely to develop such a disorder. About 10% to 30% of bvFTD is due to specific genetic causes.”

In addition, diagnosing the disease is mostly a process of elimination.

“There is no single test for frontotemporal dementia,” says Salzman. “We look for signs and symptoms of the disease and try to exclude other possible causes. Often the use of brain MRI or PET scan of the brain is useful.”

Treating FTD

Currently, there are no medical treatments available to either cure or slow down the progression of FTD. “The best we can do is prescribe medications to treat the symptoms,” explains Salzman.

Life expectancy is typically six to eight years after diagnosis, but many FTD sufferers do live longer than that.

Experts say managing symptoms and keeping the sufferer as busy and engaged as possible should be the goal for loved ones.

Dr. Henry Paulson, a professor of neurology and director of the Michigan Alzheimer’s Disease Center at the University of Michigan, believes FTD sufferers can still enjoy a high quality of life — and urges them to do so.

“I tell all my patients, ‘Don’t let this disease own you. You own it,’” he said in a CNN interview. “Sure, you’ve lost some skills because of your illness; but you still have lots of skills left and you work with the skills you have.”

This article originally appeared on Palm Beach Post: The top thing Bruce Willis' family is doing in his battle with dementia