Primary Progressive Aphasia (PPA)

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Hello everybody, welcome back to a new episode of The Truth Lies and Alzheimer's Show. I'm Lisa Skinner, your host. Once upon a time, aphasia was considered to be a symptom of dementia caused by damage occurring in our brains due to one of the over 100 brain diseases known today. Apparently, something's changed. I was actually really surprised when I heard Bruce Willis's family announced through the media that he had been diagnosed, and this was his primary diagnosis, with primary progressive aphasia. Because in the 30 years that I've been working in this industry, that is the very first time I have ever heard of anybody being diagnosed and their primary diagnosis was primary progressive aphasia or PPA. Usually people are diagnosed with like frontotemporal dementia with primary progressive aphasia, but that was his primary diagnosis. So I was a little Surprised? So, turns out the truth of the matter is that aphasia is not a symptom of dementia, but it can be a subset of frontotemporal dementia. And while frontotemporal dementia primarily affects language and behavior, aphasia is a language disorder that can occur. As a symptom of certain types of dementia, such as primary progressive aphasia, or PPA. So it is considered a type of frontotemporal dementia that starts with language difficulties and can progress to other areas of the brain. However, not all individuals with dementia will develop significant aphasia, and not all people with aphasia have dementia. Early recognition and diagnosis are crucial for effective management and support. Now, regarding Bruce Willis, he was initially diagnosed with primary progressive aphasia, aka PPA,, but then we were told that it had progressed to frontotemporal dementia, aka FTD. So that's what we're talking about today. I want to clear up this confusion and explain the differences to you. So here we go. Primary progressive aphasia, or PPA, is a neurodegenerative disease that affects our ability to speak, read, write, and understand language. Neurodegenerative diseases gradually damage parts of our brain, and they do get worse over time. If you have PPA, you may notice it's difficult to find the right words to express yourself, or you may have trouble understanding direction. Over time, you may not be able to read the newspaper, write your name, or comprehend what your loved ones are saying to you. So I guess what they're— how they're defining PPA, or primary progressive aphasia, is that it is a type or subset of frontotemporal dementia. These are a group of conditions that cause the frontal and temporal lobes of our brain to lose their function over time. These lobes are responsible for speech and language, among other functions. Primary progressive aphasia symptoms vary based on type. They affect your ability to move, to read, to speak, to understand language, and to write. And symptoms usually develop between the ages of 50 and 70 and then progressively worsen over time. Generally, there are two sub-diagnoses according to Dr. Paul Paul Barton Rosenberg, a professor of psychiatry and behavioral sciences at Johns Hopkins School of Medicine, and he says, one is a behavioral variant where people can have a change in their personality and lose their inhibitions and social graces. And then he goes on to say, another is primary aggressive aphasia, where people have trouble finding words or expressing themselves. Well, the Willis family has said that unfortunately, challenges with communication are just one symptom of the disease that Bruce has been facing. So here are symptoms and causes of frontotemporal dementia. It results from a buildup of proteins in the brain which can damage and shrink the frontal and temporal lobes in the front of your head. Because these areas of the brain are associated with personality, behavior, and language, the symptoms of a particular case of frontotemporal dementia vary depending on which area is most affected, according to Dr. Greg Day, who's a neurologist at the Mayo Clinic's campus in Florida. He says difficulty with language and understanding, as well as misinterpreting instructions, could be symptoms of frontotemporal dementia. But when the proteins build up in parts of the brain that governed social cognition or unusual behavior such as acting out, disrespecting loved ones, losing empathy, motivation, and understanding. These can all be symptoms as well. Abnormal motor functions such as problems with balance, vision, or moving one side of the body can also be symptoms. So overall, many symptoms of FTD or frontotemporal dementia can be hard to recognize in patients, and the behavioral ones in particular can be difficult to separate from psychiatric diseases, he also says. So who is most likely to get frontotemporal dementia? Well, this type of dementia tends to present in younger people, specifically those in their 40s, 50s, and 60s, the two experts said. Unlike Alzheimer's disease, which typically shows up starting at the age of 65, this disease, however, is seen a little earlier in life, which can make it harder because it's an age where people have a lot of demands and responsibilities. Dr. Day said. They might have full-time jobs, partners, or still be caring for young kids. And when it affects language for someone who relies on it making a living, it'll make a huge impact. Now, frontotemporal dementia is considered to be relatively rare, with an estimated lifetime risk of 1 in 742. Now, this is according to a 2019 study by the Mayo Clinic. They estimate that frontotemporal dementia is the cause of approximately 10 to 20% of dementia cases, making it rarer than Alzheimer's disease. Dementia refers to several degenerative disorders of the brain that affect cognition. Now, aphasia refers to difficulty expressing or understanding words due to the brain damage. And as we've talked about before, dementia is really an umbrella term that refers to several degenerative conditions affecting the brain, including Alzheimer's disease. Common symptoms across types of dementia include memory loss, confusion, personality changes, and a host of other cognitive decline. Aphasia involves a decreased ability to read, write, and speak. This is typically related to damage to one or more of the brain's language centers. And this damage can be the result of a stroke or a traumatic brain injury. So here's a closer look at the relationship between dementia and aphasia. Some of the main differences between them are that dementia symptoms can be very broad. Affecting someone's memory, personality, and behavior. And the symptoms do tend to come on gradually over time. Whereas aphasia involves specific difficulties with language. People with this condition have difficulty reading, writing, speaking, and understanding the words of others. And there are several types of aphasia. For example, conduction aphasia involves repeating words without understanding their meaning. Broca's aphasia involves choppy speech, often with a limited vocabulary. Symptoms of aphasia typically do appear after a stroke or a head trauma. So while dementia and aphasia are different conditions, dementia can cause a specific type of aphasia called primary progressive aphasia. Here we are, or PPA. Symptoms of PPA tend to develop more gradually than just regular aphasia caused by a stroke or head trauma and get worse over time. Early on, communication trouble might be minor and limited to occasionally forgetting the right word to use. As PPA progresses, it can cause a complete loss of communication abilities. PPA is a condition caused by a neurological disease that gradually affects a person's ability to use and understand language. Sometimes speaking is also extremely difficult. PPA begins slowly and worsens over time. This is different from regular aphasia from stroke or injury. Many people with PPA say, "I can't remember words," or, "I can't put sentences together very well anymore." It's helpful to think of PPA as a progressive access issue. It becomes harder to find the words, get sentences, form sentences, and sounds that were once easy for that person to use. There are different types called variants of PPA, and each PPA variant is associated with vocal problems in different parts of language, of the language network. The three main types of PPA are the semantic variant, the non-fluent or agrammatic variant, and the logopenic variant. With the non-fluent or agrammatic variant, a person's speech may be slower than before, or more monotone than before. Their speech may be effortful, halting, or mispronounced. Their sentences may be missing small connecting words like "and," "uh," or "the." Words might be missing important grammar parts like -ing, as in talking, or -ed as in talked. And understanding sentences with a lot of grammar can be extremely difficult for them as well. Now, with the semantic variant of PPA, word meaning is gradually lost. Remembering names and the meaning of words is difficult. Reading and spelling words that don't look how they sound may also be difficult. And recognizing objects and/or faces of people that you already know may become extremely hard too. And then finally, with logopenic variant of PPA, a person will have difficulty finding words. They'll have trouble repeating sentences. They'll switch or miss sounds within words when speaking. PPA is one of the clinical syndromes that fall under the umbrella of frontotemporal dementia, and unlike Alzheimer's, early memory and spatial skills are often spared. Alzheimer's disease is again the most common cause of dementia overall. And in PPA, Alzheimer's is the most often linked to logopenic variant PPA. Unlike frontotemporal dementia, memory and spatial skill problems may start to appear earlier. And then there are stages of PPA. Stage 1, which is a very mild stage. This is where individuals may notice slight difficulties with speech and language, which might be mistaken for normal aging or stress. A diagnosis is rarely made at this stage. Stage 2, or mild. This is where more frequent problems with speech and language become apparent, affecting daily activities. Family members may also notice these changes. A diagnosis of PPA is typically possible at this stage. Stage 3, or moderate. This is where communication and social interactions become increasingly challenging. Individuals may require assistance from local services and support network to manage their daily tasks. There's a Stage 4, or a severe stage, where language comprehension and communication are significantly impaired. Individuals may need help with daily living activities and may not be able to live independently at this stage. And of course, safety now becomes a primary concern. There's a Stage 5, which is called very severe, and this is where communication and understanding of language are now nearly impossible. Individuals may exhibit behavioral and cognitive changes and will now require full-time care. And then finally is Stage 6, And it's referred to as the profound stage. Individuals can no longer speak or understand language and may only produce nonverbal sounds. They may also lose their ability to move independently. So, bottom line, understanding these stages and variants can help in managing the condition and providing appropriate support for individuals affected by PPA. Early diagnosis and intervention can also play a crucial role in maintaining quality of life as the disease progresses. So now you have another thing to bring up with your physician. The conclusion is that primary progressive aphasia is a challenging condition that affects communication abilities, impacting both individuals and their families and caregivers. And once again, early diagnosis and supportive therapies can and will help manage symptoms and improve improve quality of life for those affected. So there you have it. I hope that that was helpful for you to understand the difference between aphasia and primary progressive aphasia and dementia. And that's going to conclude this episode for The Truth Lies in Alzheimer's Show. Once again, I'm Lisa Lisa Skinner, your host. If you have a chance, please, um, visit our website because it's been completely redone, and it's mindingdementia.com. So hopefully you will like the changes that you will see. And if you'd like to, I always want to remind people to subscribe to our newsletter. Because it's really a good resource for everybody and it's free. We send it out every week and it contains updated information, strategies, tips, a lot of things that you may find helpful. So as always, I wish you a great rest of your week, for all of you to stay happy and healthy, and I'll be back next week with another episode of The Truth, Lies Alzheimer's Show. Take care for now. Bye-bye.

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