Primary Progressive Aphasia (PPA)

Lisa, 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. Neuro

degenerative 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 directions 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. Him,

generally, there are two sub diagnoses, according to Dr 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.

Then he goes on to say another is primary progressive 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 govern 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 one

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 focal 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 logo panic 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 logo panic variant of PPA, a person

will have difficulty finding words and. 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 logo panic variant PPA, unlike Frontotemporal

dementia, memory and spatial skill problems may start to

appear earlier. And then there are stages of PPA, stage one,

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 two 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 three 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 four, 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

five, 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 six, 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 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 and Alzheimer's

show. Once again, I'm Lisa Skinner, your host. If you have

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truth lies and Alzheimer's show, Take care for now. Bye, bye.