Primary Progressive Aphasia (PPA)

Primary progressive aphasia (PPA) is a neurodegenerative disease that affects your ability to speak, read, write and understand language. Neurodegenerative diseases gradually damage parts of your brain. They 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.

PPA is a type of frontotemporal dementia (FTD) or Alzheimer’s dementia. These are a group of conditions that cause the frontal or temporal lobes of your brain to lose their function over time. These lobes are responsible for speech and language, among other functions.

It can be a challenging and frustrating condition to live with. It can also emotionally affect caregivers or loved ones who help those diagnosed with this condition. A healthcare provider can help you manage symptoms.

Primary progressive aphasia types

There are three types:

• Logopenic variant primary progressive aphasia: This is most often linked to Alzheimer’s disease and considered an atypical form of it. In some cases, it can also be caused by frontotemporal dementia. You may have trouble choosing the right words, naming objects and have hesitant speech with intact grammar.
• Non-fluent (agrammatic) variant PPA: It’s a type of frontotemporal dementia. It may cause poor grammar or difficulty talking fluently.
• Semantic variant: This is a type of frontotemporal dementia. You may have fluent speech, but lack comprehension. You may have trouble naming objects or understanding the meaning of stand-alone words.

Primary progressive aphasia symptoms

Primary progressive aphasia symptoms vary based on the type. They affect your ability to:

• Move
• Read
• Speak
• Understand language
• Write

Symptoms usually develop between ages 50 and 70. They get worse over time.

Logopenic variant symptoms

Symptoms of the logopenic variant may include:

• Difficulty finding the correct word to say
• Difficulty understanding long or complex sentences
• Finding longer sentences or phrases harder to say than shorter ones
• Making frequent pauses while speaking
• Making mistakes with speech or having trouble pronouncing all of the sounds
• Using substitutions for common words (like dog instead of cat)

Non-fluent (agrammatic) variant symptoms

Symptoms of the non-fluent (agrammatic) variant may include:

• Difficulty moving your lips and tongue to make speech sounds (apraxia)
• Difficulty swallowing
• Movement challenges (slow and stiff movements, a loss of balance or frequent falls)
• Removing words from sentences to keep phrases simple (like “go dinner mom” instead of “I’m going to dinner with my mother”)
• Trouble understanding long or complex sentences

You may not feel comfortable speaking at all (mutism) if you have non-fluent PPA.

Semantic variant symptoms

Symptoms of the semantic variant may include:

• Difficulty remembering what familiar objects are and what they’re used for
• Problems remembering what words mean
• Trouble finding the right word to say
• Trouble reading and writing

Primary progressive aphasia causes

This condition develops when the parts of your brain that control language start breaking down. This happens in your frontal and temporal lobes (depending on the type). Your brain tissue shrinks (atrophies), affecting your ability to communicate and understand language.

Risk factors for PPA

This condition may affect anyone. You may be more at risk if you have:

• A biological family history of PPA
• A genetic variant (C9orf72, MAPT or GRN)
• A learning disability
• Alzheimer’s disease

Sometimes, it may happen for no known reason.

Complications

As PPA gets worse, you may develop symptoms that affect your:

• Memory
• Emotions
• Judgment
• Personality
• Thinking

This can make performing everyday tasks difficult or unsafe. It can also lead to mental health challenges, like depression or anxiety.

How doctors diagnose primary progressive aphasia

A healthcare provider will diagnose primary progressive aphasia after a physical examination and testing. They’ll ask questions to learn more about your medical history and known biological family history to see if you have an increased risk of PPA.

Testing may include:

• Neurological examination
• Cognitive tests
• Imaging tests of your brain (MRI or CT scan)

PPA stages

Your healthcare provider may diagnose primary progressive aphasia in stages. Stages of PPA include:

• Stage 1 (very mild): You may notice a few difficulties with speech and language. It may seem like a normal part of aging or stress. A diagnosis rarely happens this early.
• Stage 2 (mild): You notice more frequent problems with speech and language. Your loved ones may notice, too. It starts to affect your daily activities. By this stage, a diagnosis of PPA by an experienced clinician should be possible.
• Stage 3 (moderate): Many aspects of your daily life are challenging to do on your own. You may find communication and socializing very frustrating. Getting help from local services and support networks is important.
• Stage 4 (severe): Communication and understanding language are very difficult. You may need assistance with daily living tasks or you may not be able to safely live independently. At this stage, safety is the main concern. It’s also important to support caregivers to prevent stress and burnout.
• Stage 5 (very severe): Communication and language understanding are nearly impossible. You may have behavior and cognitive changes. You’ll need full-time care.
• Stage 6 (profound): You can’t speak or understand language. You may make non-verbal sounds. You may lose the ability to move independently.

Your provider will keep track of your symptoms, noting how they progress. The stage may change as the condition affects more of your brain.

How is primary progressive aphasia treated?

There isn’t a cure for primary progressive aphasia. There’s currently no way to stop it from getting worse. But some treatments may slow the disease’s progression or increase your quality of life.

Your provider may recommend the following treatment options for PPA:

• Participating in speech therapy
• Learning new ways to communicate, like sign language
• Taking medicines, like selective serotonin reuptake inhibitors (SSRIs), to manage behavioral changes and reduce depression and anxiety
• Taking Alzheimer’s medications, if it’s the cause and your provider recommends them (may include new anti-amyloid infusion treatments or symptom-relief medicines, like donepezil or rivastigmine)

When should I see my healthcare provider?

Visit a healthcare provider if you have trouble with your speech or language skills. Let them know if these symptoms get worse and make it difficult for you to do certain activities. Some examples might include:

• You have trouble finding the right word to say.
• You mix up words in a sentence.
• You can’t follow conversations.
• You have trouble reading or writing.

What can I expect if I have primary progressive aphasia?

Primary progressive aphasia worsens over time. You may eventually lose your language and communication skills after many years. As the condition affects more of your brain, it will also affect your thinking and behavior. This may make living independently unsafe. Your care team will be available to help you as the condition gets worse.

It’s important to discuss this diagnosis with your loved ones as early as possible. You can make a plan to file an advance directive. This document lets your loved ones know how to care for you when you’re unable to care for yourself. If you’re unsure where to start, talk to a healthcare provider.

How long does someone live with PPA?

The life expectancy for primary progressive aphasia varies from person to person. Many people live for several years after diagnosis, often around five to seven, and sometimes longer from when symptoms first appear.

The best person to talk to about your situation is your provider. Your case may or may not match statistics.