Primary progressive aphasia is a rare type of aphasia that is caused by brain atrophy. It’s associated with dementia. While there’s no cure, treatment can help with its progression.

woman with primary progressive aphasia receiving treatment from a healthcare professionalShare on Pinterest
fotografixx/Getty Images 165997999

Aphasia is the medical term for conditions that cause difficulty with speech and language. Typically, aphasia is caused by damage to the brain, either from injury or from a medical crisis such as a stroke.

Most types of aphasia can be treated, and recovery is often possible. However, there is currently no cure for a rare type of aphasia called primary progressive aphasia.

This type of aphasia is caused by brain atrophy and continually gets worse over time. Treatments such as speech therapy can help maintain communication skills and slow down the progression of symptoms, but they cannot cure this type of aphasia entirely.

Primary progressive aphasia is a rare condition that causes communication difficulties. It’s linked to frontotemporal dementia and Alzheimer’s disease.

Primary progressive aphasia affects the nervous system and causes slow and progressive changes in communication abilities over time. People with this condition lose the ability to speak, write, and understand both written and spoken language.

There are three subtypes of primary progressive aphasia:

  • Semantic primary progressive aphasia (svPPA): This form of primary progressive aphasia causes increased difficulties recalling facts, as well as the names of both people and objects.
  • Nonfluent/agrammatic primary progressive aphasia (nfvPPA): This type leads to increased difficulty pronouncing words and sounds.
  • Logopenic primary progressive aphasia (lvPPA): This type causes difficulties in finding the right word.

The symptoms of primary progressive aphasia depend on the subtype and on the severity. Since this condition is progressive, symptoms are typically mild at first but can become more severe after a few years.

Symptoms of primary progressive aphasia include:

  • difficulty reading
  • not understanding spoken language
  • forgetting the meanings of certain words
  • not being able to name certain objects
  • difficulty using grammar in writing
  • difficulty using grammar while speaking
  • difficulty understanding long or complex sentences
  • confusing letter or word sounds when speaking
  • frequently pausing while talking
  • difficulty finding the right words to communicate needs and thoughts

Primary progressive aphasia occurs when the sections of the brain that are responsible for speech and language shrink. This is called brain atrophy, and it is linked to the presence of abnormal brain proteins.

More research into the exact reasons some people develop primary progressive aphasia and other conditions related to brain atrophy, such as dementia and seizure disorders, is still needed.

A diagnosis of primary progressive aphasia will begin with a review of symptoms.

A healthcare professional will want to discuss changes in your communication and thinking patterns and will want to know how long those changes have been present. They might also order testing to help rule out other conditions and to confirm the diagnosis of primary progressive aphasia.

Tests might include:

  • Blood tests: Blood tests can look for infections that cause similar symptoms. They can also look for genetic changes that are sometimes linked to neurological conditions, such as primary progressive aphasia.
  • Speech-language evaluation: A speech-language evaluation can measure your speech and language skills against objective standards.
  • Neurological tests: Neurological tests will measure your ability to recall facts, name objects, and more.
  • Brain scan: A brain scan can show brain atrophy. It can also detect visible signs of other conditions that could cause similar symptoms. The scan might be done with an:
    • MRI
    • single-photon emission computerized tomography (SPECT) scan
    • PET scan

Therapy can help you manage primary progressive aphasia and slow the progression of your symptoms. Speech and language therapy is the primary form of treatment.

During speech and language therapy, a speech therapist will guide you through communication exercises and will give you the tools you need for daily life with primary progressive aphasia.

Sometimes, communication devices and other aids are helpful for people with primary progressive aphasia. Your speech therapist will help you find what works best for you.

There’s currently no cure for the condition.

For most people with primary progressive aphasia, the cause is unknown. However, there are a few known risk factors, including:

  • Inherited genetic mutations: Primary progressive aphasia appears to have a genetic link. If a member of your family has primary progressive aphasia, your risk for developing the condition is higher.
  • Learning disabilities: Childhood learning disabilities, especially developmental dyslexia, slightly increases the risk for primary progressive aphasia.

The early stages of primary progressive aphasia generally lasts around 2 years. During this time, people with primary progressive aphasia will experience a loss of communication and language skills but typically won’t see a change in cognitive or functional skills.

In later stages, primary progressive aphasia begins to affect cognition, memory, and vision. People in later stages are typically no longer able to care for themselves. The speed of progression can vary from person to person.

Some research suggests the subtype of primary progressive aphasia can have an effect on life span after diagnosis. However, primary progressive aphasia is rare, and sample sizes are small.

A 2021 study analyzed life span data across progressive primary aphasia subtypes. Researchers found that additional factors, such as region and age at diagnosis, could be further influencing these results.

You can learn more about primary progressive aphasia by reading answers to some common questions.

When are people typically diagnosed with primary progressive aphasia?

It’s most common to be diagnosed with primary progressive aphasia when you’re between the ages of 50 and 70.

How is primary progressive aphasia different from aphasia after a stroke or brain injury?

Primary progressive aphasia is a progressive condition without an exact cause. Speech therapy can help slow down the progression of symptoms, but brain atrophy in the cases of primary progressive aphasia will continue.

On the other hand, it’s possible to recover from aphasia that’s a result of a stroke or brain injury. As your brain heals, speech therapy can help rebuild connections that are responsible for communication and language.

Are there medications for primary progressive aphasia?

There are currently no medications that can treat the symptoms of primary progressive aphasia. However, your doctor might prescribe medications to help treat complications or common co-occurring conditions.

For example, some people with primary progressive aphasia experience mood disorders such as anxiety and depression and are prescribed medications for them.

Primary progressive aphasia is a condition caused by brain atrophy that results in a loss of communication and language skills. The condition is rare and is linked to dementia and Alzheimer’s disease.

Symptoms can vary depending on the subtype of primary progressive aphasia and will get worse as the condition progresses. Common symptoms include difficulty expressing thoughts, finding words, understanding others, naming objects, reading, and using complete sentences.

There is no cure for primary progressive aphasia, but speech therapy can help slow down the progression of symptoms.