Primary Progressive Aphasia (PPA)

Overview

What is primary progressive aphasia?

Primary progressive aphasia (PPA) is a neurological disorder that affects a person’s ability to speak and communicate. Aphasia is a language disorder that results from damage to key parts of your brain that are responsible for understanding or producing speech and/or writing.

When the aphasia is from a brain disease that gets worse over time (progressive neurodegenerative disease), healthcare providers call it “primary progressive aphasia.” Initially, people may have trouble finding the right words to express themselves. The condition worsens over time, as people lose their ability to write, speak or understand language.

Most commonly, the underlying cause of PPA is a neurodegenerative disease like Alzheimer’s disease or frontotemporal dementia. In these conditions, the areas of your brain related to control of speech or understanding of words and their meaning break down.

What are the types of primary progressive aphasia?

Experts divide PPA into three sub-types:

  • Lopogenic progressive aphasia may cause difficulty finding the right words or understanding others.
  • Progressive non-fluent aphasia may cause poor grammar or difficulty talking fluently.
  • Semantic dementia may cause difficulty naming objects or understanding the meaning of stand-alone words.

Who does primary progressive aphasia affect?

Primary progressive aphasia may affect anyone, but it’s more common in people with:

  • Family history of PPA.
  • Gene changes (mutations) in the GRN gene, which occur before birth.
  • Learning disabilities.

Symptoms and Causes

What are the symptoms of primary progressive aphasia?

People usually develop PPA symptoms between ages 50 and 70. The condition may start as minor speech problems. Over time, people may struggle with judgment and thinking.

Primary progressive aphasia symptoms may include:

  • Difficulty finding the correct word for an object consistently.
  • Frequent pauses while speaking.
  • Poor grammar.
  • Slow speech.
  • Trouble understanding speech.
  • Total loss of language skills.

What causes primary progressive aphasia?

Primary progressive aphasia develops when the parts of your brain that control language start breaking down. Your brain tissue shrinks (atrophies), affecting your ability to communicate. Sometimes, this breakdown starts because of a gene mutation that you’re born with. Other times, it may happen for no known reason.

In most people, healthcare providers often can’t identify a clear predisposing risk factor. A combination of environmental and genetic factors could cause PPA. Even if you don’t have a gene mutation, PPA may still run in your family.

Diagnosis and Tests

How is primary progressive aphasia diagnosed?

Your healthcare provider may suspect PPA based on your symptoms. They’ll evaluate your medical history and family history to see if you have an increased risk of primary progressive aphasia.

You might have specialized cognitive tests and brain scans, such as an MRI or CT scan, to confirm a diagnosis.

Management and Treatment

How is primary progressive aphasia treated?

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

People may benefit from:

  • Participating in speech therapy and cognitive therapy can help to maintain language and thinking skills for as long as possible.
  • Learning new ways to communicate, such as sign language.
  • Taking medicines, such as selective serotonin reuptake inhibitors (SSRIs) to manage behavioral changes and reduce anxiety or depression associated with PPA.
  • Taking medications approved for Alzheimer’s disease, if that’s the underlying cause of PPA.

Prevention

How can I reduce my risk of primary progressive aphasia?

There’s no guaranteed way of preventing primary progressive aphasia. Some lifestyle changes may reduce your risk of developing dementia, such as:

  • Achieving and maintaining an ideal weight for your age, sex and body type.
  • Avoiding serious head trauma by wearing your seatbelt and reducing your risk of falls.
  • Eating a balanced diet full of fruits, vegetables, whole grains, lean protein and healthy fats.
  • Exercising regularly, incorporating both aerobic activity and strength training.
  • Limiting daily alcohol intake to two drinks or fewer for men and one drink or fewer for women.
  • Maintaining strong social connections.
  • Managing your heart health by keeping your blood pressure and cholesterol within healthy ranges.
  • Quitting smoking.

Outlook / Prognosis

What can I expect if I have primary progressive aphasia?

Primary progressive aphasia worsens over time. Many people with PPA eventually lose their language skills over many years, limiting their ability to communicate.

Most people who have the condition live up to 12 years after their initial diagnosis. Eventually, many people need daily support with their usual activities.

Living With

What questions should I ask my doctor?

If you or a loved one has suspected or diagnosed primary progressive aphasia, you may also want to ask your healthcare provider:

  • What are the early signs of primary progressive aphasia?
  • What tests diagnose primary progressive aphasia?
  • Are there treatments to slow the progression of primary progressive aphasia?
  • What can I do to increase my quality of life with primary progressive aphasia?

A note from Cleveland Clinic

Primary progressive aphasia (PPA) is a gradual loss of language skills. It’s a sign of an underlying neurodegenerative disease. In some people, PPA is the first sign of Alzheimer’s disease, while in others, it’s related to frontotemporal dementia. Experts don’t always know what causes this condition, but it’s often a combination of environmental and genetic factors. There isn’t a way to reverse primary progressive aphasia, but treatment may help you maintain communication skills for as long as possible. Talk to your healthcare provider about the best treatment for you.

Last reviewed by a Cleveland Clinic medical professional on 04/12/2022.

References

  • Alzheimer’s Association. Can Alzheimer’s Disease Be Prevented? (https://www.alz.org/alzheimers-dementia/research_progress/prevention) Accessed 4/12/2022.
  • Alzheimer’s Research UK. Primary progressive aphasia risk factors. (https://www.alzheimersresearchuk.org/dementia-information/types-of-dementia/primary-progressive-aphasia/risk-factors/) Accessed 4/12/2022.
  • Genetic and Rare Diseases Information Center. Primary progressive aphasia. (https://rarediseases.info.nih.gov/diseases/8541/primary-progressive-aphasia) Accessed 4/12/2022.
  • National Aphasia Association. Primary Progressive Aphasia. (https://www.aphasia.org/aphasia-resources/primary-progressive-aphasia/) Accessed 4/12/2022.

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