Lab Probes Aphasia, a Language Disorder That Struck Bruce Willis, Wendy Williams

Following the high-profile announcement of Bruce Willis’s diagnosis of primary progressive aphasia, now talk show host Wendy Williams has been diagnosed with the same illness. What are the types of FTD aphasia?

A primary progressive aphasia just signifies that the aphasia – the loss of words or acquired loss of words – is the primary problem. And it slowly gets worse over time. This signifies that it’s due to a neurodegenerative illness. It’s one of the many diseases that slowly progresses, causing a loss of what our brain usually does. Among those functions is language. There are classically considered to be three forms of PPAs.

Usually it’s on the left side since that’s the side of the brain that's shrinking, since that side helps us to communicate using language. Two of the PPAs are considered frontotemporal dementias, meaning that the part of the brain that is shrinking is either frontal or temporal. The other type of primary progressive aphasia involves a farther-back part of the brain – the temporo-parietal junction. This third type is due to Alzheimer’s pathology and it’s presenting in an unusual way. In this case, the Alzheimer’s disease is causing language loss rather than causing memory problems.

The two frontotemporal forms are usually not due to Alzheimer’s disease. Rather, they are associated with other proteins that misfold or are deposited in the brain, resulting in shrinkage of either the frontal or temporal lobes. Each of these three forms of aphasia cause different types of language disorders. So, we can often predict what we would see in an MRI with a brain shrinkage or what we’d see under a microscope with a protein based on the type of language problem someone is having.

In what age range do patients typically begin to experience symptoms?

Typically, it’s people who are middle-aged to elderly. Again, for reasons we don’t understand. It tends to be an onset at younger ages than typical Alzheimer’s cases.

Could you elaborate on how something besides a neurodegenerative disorder, such as a brain injury or a stroke, can result in different types of aphasia?

Yes, different aphasias can be caused by different things. So, an injury to the head and part of the brain that is important for speech can cause that. Some of the earliest (age-wise) cases of aphasia were likely due to stroke. So that’s Broca’s (affects language production) and Wernicke’s (affects language comprehension) aphasias.

For some reason that we don’t understand, when the aphasia is due to that sort of sudden injury, like a stroke or traumatic brain injury, the symptoms are a little different, even if it’s in the same location as a primary progressive aphasia. We don’t know why that is, frankly. I wish I could tell you.

What are the treatments that are available to people who develop an aphasia?

So, there are different types of speech therapies that are available, and that speech therapy should be tailored to the specific type of aphasia. So, if you have what they call a non-fluent aphasia, which causes difficulty with pronunciation, for example, the speech therapy might focus more on ways to pronounce words. Whereas, if you have a different type of aphasia – the semantic variant, which impacts the temporal lobe, you might focus more on exercises of word meaning rather than just the movements required to produce speech.

In a primary progressive aphasia, there’s that daunting word “progressive.” It’s going to worsen over time (and there is no current cure).

In other forms of aphasia, such as a change in language due to a traumatic brain injury, you may be able to have more recovery and you may be able to better cope with word changes throughout the rest of your natural lifespan. So, it depends once again on the cause of the aphasia.

Can you describe one of the projects you’re currently working on in your lab?

One of the things we’re looking at are automated measures of assessing communication. So, how much can we learn just from spontaneous communication, for example? We are looking not only at primary progressive aphasias, but also more common neurocognitive disorders like Alzheimer’s. We’d like to not only predict if somebody has a neurocognitive disorder, but also better understand the severity of their disease and other symptoms that might be going on in that disease course. This uses a lot of computational AI techniques. We’ll be presenting some early results at the upcoming American Academy of Neurology in April.

It would seem that the gradual loss of language – either expression or comprehension – in patients with primary progressive aphasia would cause worsening frustration, even to the point the patient may begin to avoid social interactions. Do you find that these patients move toward a more socially isolated existence?

It depends a lot on the individual, but also on their environment. Some people do get embarrassed and withdraw more. That’s pretty common. However, other folks are able to find support systems that allow them to still get along well with other people. These may allow them to still communicate their needs using maybe alternative forms of communication, or just comfortably being with other people without communicating as much.

One of the nicer things about a primary progressive aphasia – and we look for silver linings in this work – is that while nobody would wish to have it, many other aspects of cognition are preserved. And so, there are instances of people who, for example, build a cabin for themselves despite having a limited ability to speak. So, if patients focus on what they can do with these conditions, rather than getting hung up on and frustrated by the things they can’t, they tend to have higher qualities of life.