Primary Progressive Aphasia

What is Primary Progressive Aphasia (PPA)?

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 difficult. PPA begins slowly and worsens over time. This is different from aphasia from stroke or injury. Many people with PPA say: “I can’t remember words” or “I can’t put sentences together well anymore” It’s helpful to think of PPA as a progressive “access” issue — it becomes harder to get to words, sentences, and sounds that were once easy to use.

There are different types (called “variants”) of PPA. Each PPA variant is associated with focal problems in different parts of the language network. The three main types of PPA are the semantic variant, nonfluent/agrammatic variant, and the logopenic variant (see What is Primary Progressive Apraxia of Speech? for information about this related problem).

What Causes Primary Progressive Aphasia?

PPA is caused by a gradual loss of function within brain cells in the areas of the brain that control language. These changes happen slowly and get worse over time. These changes happen because of disease inside brain cells. Eventually, those parts of the brain shrink away (or “atrophy”). Semantic variant PPA and nonfluent/agrammatic variant PPA are most commonly caused by Frontotemporal Degeneration (FTD). Logopenic variant is most commonly caused by Alzheimer’s-related plaques and tangles.

Types of PPA

Image of the brain circled to show part of the brain effected by Nonfluent/Agrammatic PPA

Nonfluent/Agrammatic Variant PPA (nfvPPA)

Speech may be slower than before, or more monotone than before
Speech may be effortful, halting or mispronounced
Sentences may be missing small connecting words, like “and” “a” or “the”
Words might be missing important grammar parts like -ing (as in “talking”) or -ed (as in “talked”)
Understanding sentences with a lot of grammar can be hard too.

Learn more about Nonfluent/Agrammatic PPA

Brain image with circle indicating location effected by semantic Variant PPA

Semantic Variant PPA (svPPA)

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 be difficult.
Recognizing objects and/or faces of people you know may be hard too.

Learn more about Semantic PPA

Brain image showing the part of the brain effected by logopenic PPA

Logopenic Variant PPA (lvPPA)

Difficulty finding words
Trouble repeating sentences
Switching or missing sounds within words when speaking

Learn more about Logopenic PPA

Frontotemporal Degeneration (FTD) and Alzhiermer’s-Related Plaques and Tangles

Frontotemporal Degeneration (FTD)

FTD is a group of brain diseases that affect the frontal and/or temporal lobes of the brain. In PPA, the disease affects areas responsible for speech and language. FTD is the most common cause of dementia before the age of 60, but can happen as young as the 30s and as late as the 80s.

FTD is the most common cause of dementia for people under 60.
PPA is one of the clinical syndromes that fall under the umbrella of FTD.
Unlike Alzheimer’s, early memory and spatial skills are often spared.

For more: The Association for Frontotemporal Degeneration

Alzheimer’s-Related Plaques and Tangles

Alzheimer’s is a disease caused by buildup of bad proteins in and around brain cells. In one type of PPA, they build up in areas used for language. Two key proteins are involved: amyloid plaques and tau tangles. These proteins build up slowly over time, leading to brain cell damage and loss.

Alzheimer’s Statistics

Alzheimer’s disease is the most common cause of dementia overall.
In PPA, Alzheimer’s is most often linked to logopenic variant PPA.
Unlike FTD, memory and spatial skill problems may appear earlier.

How Is PPA Diagnosed?

Diagnosing PPA usually takes a team. You may see several doctors, neuropsychologists, and speech-language pathologists. You may get brain scans to help figure out the problem.

Common steps include:

Explaining what’s been going on to your medical team.
Tests of speaking, understanding, reading, and writing.
Testing other brain functions like body movements and memory to make sure it’s only a problem with language.
Brain scans: MRI or PET scans to view changes in brain regions.
Making sure something else isn’t causing it, like problems with making and using energy in the body or problems with sleep
Spinal Tap: Some people get a spinal tap (or “biofluid test” or “cerebral spinal fluid test”) to look for certain signs of disease.

Accurate diagnosis is key. PPA is not well known by many medical providers. Many people have to go to specialty medical centers at universities before they get a correct diagnosis. Contact us for personalized guidance.

Treatment & Support

There is no cure for PPA, but speech-language treatment can help maintain skills and quality of life.

Speech-language treatment for PPA focuses on:

Getting better at words and sentences you practice
Keeping communication skills up
Finding strategies for word finding and conversation
Teaching care partners how to support communication
Exploring other ways to communicate

Learn more about speech-language treatments for PPA and communication strategies for PPA

What to Expect

PPA changes over time in different ways for different people. Talking and understanding get harder over time for everyone. The type of PPA that you have can help doctors guess what kind of problems might happen later on. But not everyone follows a typical pattern of decline. Some people have changes that do not follow the usual path.

Emotional Impact & Coping

Living with PPA can be lonely and frustrating. Both individuals with PPA and their care partners may experience grief, loss, and changes in roles.

You’re not alone. Support is available. The NAA offers:

PPA Chats for people with PPA and the people in their lives
PPA Care Partner Support Group for people who care about someone with PPA
Aphasia Ambassadors Program

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