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Wendy Williams' Medical Diagnosis: Explaining Primary Progressive Aphasia and Frontotemporal Dementia
View Date:2024-12-24 03:25:24
It's been more than two years since Wendy Williams' stepped away from her beloved daytime talk show as she battled various health conditions.
She's made a few public appearances since, but in April 2023 she entered a facility to be treated for "cognitive issues," as her manager Will Selby said in a scene from the upcoming Lifetime documentary Where Is Wendy Williams?.
And on Feb. 22, her team shared that the 59-year-old was diagnosed in 2023 with primary progressive aphasia—a neurological condition that affects a person's ability to process language—and frontotemporal dementia, which affects behavior and cognitive functions.
Their statement called the decision to disclose her condition "difficult" and carefully considered, but they wanted to "correct inaccurate and hurtful rumors about her health."
"Over the past few years," the statement continued, "questions have been raised at times about Wendy's ability to process information and many have speculated about Wendy's condition, particularly when she began to lose words, act erratically at times, and have difficulty understanding financial transactions."
The dual conditions have already "presented significant hurdles in Wendy's life," they noted, but she is "still able to do many things for herself. Most importantly she maintains her trademark sense of humor and is receiving the care she requires to make sure she is protected and that her needs are addressed."
Naturally, the news about Williams calls to mind Bruce Willis' aphasia diagnosis, which his family first went public with in March 2022. Last February they shared that he was also living with frontotemporal dementia. And throughout this phase of their journey together his wife Emma Heming Willis and the grown daughters he shares with ex-wife Demi Moore—Rumer Willis, Scout Willis and Tallulah Willis—have shared candid updates about the now-68-year-old's increasingly debilitating condition.
To better understand what having aphasia and frontotemporal dementia means, E! News spoke exclusively to Dr. Mario F. Mendez, the Director of Neurobehavior at the VA Greater Los Angeles and Professor of Neurology and Psychiatry at UCLA, where he directs the Focal-type Dementia Clinic and Program.
What is primary progressive aphasia?
"Aphasia just means language impairment," Mendez said, "a disturbance or an impairment in the brain's use of symbols for communication."
The behavioral neurologist also clarified that aphasia is a neurogenerative disorder that's a type of dementia, namely one of the kinds known as frontotemporal dementia or frontotemporal lobe degeneration.
There are two forms of progressive language impairment, nonfluent variant and semantic variant. (Williams' team did not disclose which type she has.)
"At least half of the progressive aphasias are nonfluent," the doctor explained, "which means they have difficulty in the production of language as manifested by speech," though that could also come through in writing and other modes of communication, including sign language.
"The typical nonfluent," he continued, "they have problems with grammar and syntax. A lot of language is putting together words in a sentence, and what binds a sentence are functional words and grammatical structure."
"Patients with nonfluent PPA, they often have disturbances in the flow of their speech," Mendez said. "It's segmented and slow, sometimes the sounds are distorted and mispronounced."
Still, he added, those patients are able to understand simple sentences.
The other, "very different" form of PPA is the semantic variant, where "they lose the meaning of words," Mendez noted. "To an untrained listener they can sound quite normal, but they're losing words. They can talk around the words they can't use."
When they forget the word "table," for instance, instead they'll say something like, "Why don't you put your pens and paper on that flat surface over there?" he said. "That's another form of PPA and they're all part of these frontotemporal dementia syndromes."
Why does an aphasia diagnosis turn into aphasia and dementia?
"Dementia simply means a decline in cognition," Mendez said, noting that while the word is most commonly associated with memory loss, it applies to other manifestations of degeneration in the brain as well.
"In this case [of PPA]," he said, "the decline is in language, another aspect of cognitive processing."
And the reason why a person may be initially diagnosed with only a form of aphasia at first—the update about Willis having dementia came 11 months after his family shared he'd be retiring from acting due to aphasia—is basically because the diagnostic community collectively agreed to do it that way.
"That's how those terms are used," Mendez explained. "We call it primary progressive aphasia, or a loss of language, until it's really impairing and there are other aspects of behavior and cognition affected. Then we start using the word dementia."
How long might a person have aphasia before developing dementia?
Mendez said two years was the usual "arbitrary cutoff" for progressive aphasia remaining a person's sole diagnosis. "By then, if it's going to spread," he explained, "it usually spreads to adjacent frontal and anterior temporal regions and the person has more behavioral frontotemporal dementia."
Noting that he wasn't speaking with personal knowledge of Willis' condition, Mendez said it was possible that the actor had aphasia for a few years, "then it started to affect the adjacent parts of the brain, which is a common scenario. Not inevitable, but a common scenario."
Is there a more common age at which people are diagnosed with aphasia?
Mendez said that symptoms tend to start presenting, as observed by physicians, when a person is in their mid- to late 50s.
"Generally one of the things that's frightening about these disorders is they begin in what I call middle-life, under the age of 65," he said. "Having these illnesses come on in the prime of your life when you're productive and you've established your career and are taking care of your family... it's extremely devastating."
Are there lifestyle factors that increase the chance of getting aphasia?
Mendez said that research, to date, has not identified any environmental risk factors for these disorders that are "on the spectrum of frontotemporal dementia," i.e. disorders involving the degeneration of the frontal and anterior temporal lobes, where the areas that affect language are located.
"What turns on that process, we know in about 15 percent [of cases], is a bad gene," he said. "There are three major classes of bad genes that do that, so genetic analysis is important because it can run in families. The genes have been a window to figure out what the mechanism is for these disorders."
But, Mendez stressed, "these are relatively rare disorders, and therefore they have been underdiagnosed, misdiagnosed, missed. There are a lot of things we just don't know."
At the same time, research and clinical trials continue to get at the root of this condition in hopes of getting a handle on this currently incurable condition. Because there's no single test to tell you whether you have it, yea or nay, "we're particularly working on biomarkers," he said, referring to the biological indicator of a process (such as aging), event or condition. "There's a mechanism that is revealed by understanding how the gene manifests a disease. We hope that will be a window to finding treatments that attack these mechanisms."
Do head injuries cause aphasia and dementia?
Asked if there's link between head trauma and aphasia, Mendez put it this way: "I like to think of these neurogenerative illnesses as not occurring in a vacuum, they occur in the context of the rest of the brain. You have a certain brain potential, and the more injuries you have, the lower your cognitive reserve and your neuronal reserve. You're more susceptible to crossing into impairment with these illnesses" and there's a greater chance of developing a form of frontotemporal dementia earlier in life.
Head injuries, he said, are simply "not good for the brain."
Is there treatment for aphasia?
Assuming first and foremost that a patient has been properly diagnosed, "there's a lot of things to be done," Mendez said. "We don't have a pill that takes this away, but there are medicines we use to alter neurotransmitters, some to try to help people speak."
Some patients respond to transcranial stimulation with direct current and transcranial magnetic stimulation, the doctor continued. And there are a number of types of speech therapy, some of which involve figuring out ways to communicate other than by talking.
A course of treatment "has to be very individualized," Mendez noted, "but anything from helping them find ways to make the words com out more intelligible to using alternative communicating devices."
Very importantly, the doctor added, "you have to have mechanisms to support the caregivers."
While acknowledging that part of a loved one's brain isn't working like it used to, he explained, "if you emphasize what the person can do, what they have retained, and show them how they can get around this roadblock of language [impairment], then for a significant period of time, depending on the individual, you can really improve their lives."
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