The announcement this week that actor Bruce Willis, known for his roles in such films as “Die Hard,” “The Sixth Sense,” and “Pulp Fiction,” is retiring after being diagnosed with aphasia has brought attention to a disorder that many people haven’t heard of, even though it affects an estimated 2 million Americans.
Aphasia is not an illness, but rather a condition that occurs when the brain’s language center suffers damage, which can happen for a variety of reasons.
People with aphasia have difficulty communicating with language, whether speech or the written word. Some people with aphasia can’t express their thoughts while others can’t understand what people are saying.
By itself, aphasia doesn’t indicate cognitive loss. “It’s a loss of language, not intellect,” said Mary Ann Williams-Butler, supervisor of the speech pathology department at Emerson Hospital in Concord.
But one form of aphasia signals the start of a degenerative illness that leads inexorably to dementia, and experts suspect that, sadly, this is what Willis has.
Each year some 180,000 Americans acquire aphasia, according to the National Aphasia Association.
Most of them have suffered a stroke, brain tumor, hemorrhage, infection, or other trauma to the left frontal lobe, which processes language. People often recover, at least partially, from such injuries. Speech-language therapy can marshal unaffected parts of the brain to compensate for losses elsewhere, and the damaged brain tissue does heal, Williams-Butler said.
But another type of aphasia, known as primary progressive aphasia, or PPA, is a degenerative brain disease with no cure. PPA is a form of dementia in which loss of language is the “leading edge” of a process toward more pervasive cognitive decline over several years, said Dr. David E. Thaler, neurologist-in-chief at Tufts Medical Center. “That’s frankly what this seems like,” Thaler said, referring to what is known about Willis’s illness.
In announcing his retirement Wednesday, the 67-year-old actor’s family made no mention of stroke or other brain injury, and said that aphasia was “impacting his cognitive abilities.” Additionally, people in the film industry have noticed a deterioration in Willis’s abilities for years, according to the Los Angeles Times. He needed his lines dictated through an earpiece, the Times reported.
Primary progressive aphasia can, rarely, be the first sign of Alzheimer’s disease. Alzheimer’s usually manifests as memory loss, but sometimes language loss happens first, said Deepti Putcha, a clinical neuropsychologist at Mass General Brigham.
More commonly, though, PPA is a form of frontotemporal dementia, which occurs when brain cells die in the lobes behind the forehead and behind the ears. Abnormal deposits of two types of proteins have been linked to this form of dementia.
Frontotemporal dementia accounts for 10 percent to 20 percent of all dementia cases, affecting an estimated 50,000 to 60,000 Americans, according to the Association for Frontotemporal Degeneration. It tends to first manifest when people are in their 50s or 60s.
PPA starts slowly, and at first can resemble the normal difficulty retrieving words that many people experience as they age, Putcha said. But when the forgetfulness gets worse and starts to affect day-to-day activities, she advises that people seek an evaluation at a specialized clinic such as Massachusetts General Hospital’s Frontotemporal Disorders Unit, where she works.
Speech-language therapists teach strategies that help the patient communicate and the caretaker understand, said Williams-Butler, of Emerson Hospital.
“At any stage, we have the ability to help improve or support communication,” she said. But it’s best to start early, before there is too much cognitive decline.
In the early stages, patients can learn word-retrieval strategies that can lead to improvement, at least for a time, said Louise Kimura, a speech-language pathologist at Emerson Hospital. They can also learn how to use speech devices, such as electronic tablets that speak words out loud when the user clicks on an image.
It’s not all hopeless, Putcha said. “We don’t have treatments to stop or reverse it,” she said, “but there’s a lot we can do to optimize [the person’s abilities] and slow the decline.”