Guide to Primary Progressive Aphasia (PPA): Causes, Symptoms, and Treatment

This article is a guide to primary progressive aphasia, also known as PPA. It will help you learn what PPA is, the main symptoms of primary progressive aphasia, and how this type of aphasia is treated. 

What is primary progressive aphasia?

You may know of aphasia as a language impairment that occurs after a stroke, head injury, or tumor. Primary progressive aphasia is different. PPA is a type of dementia that involves a gradual decline in language function. Rather than occurring due to an injury to the brain, PPA results from a buildup of proteins associated with Alzheimer’s disease or frontotemporal lobar degeneration (FTLD). 

PPA is a type of dementia that involves a gradual decline in language function.

The onset of PPA usually happens between 40 and 65 years of age. PPA is a progressive disease, which means it worsens or spreads over time. It continues to affect language and eventually involves other cognitive and behavioral functions such as memory, swallowing, judgment, movement, and personality.

Primary progressive aphasia is considered fairly uncommon. There are approximately three cases per 100,000 people. 

What are the symptoms of primary progressive aphasia?

Although the symptoms of PPA will vary from person to person, the initial symptoms can include:

  • Word-finding difficulties

  • Slowed speech

  • Incomplete sentences

  • Abnormal word order when speaking or writing

  • Difficulty understanding what words mean

  • Inability to recall names of friends and family members

  • Changes in the ability to spell words

What are the types of PPA?

Primary progressive aphasia is broken down into three primary categories. These types of PPA are based on the areas of the brain with protein buildup and the difficulties that result.

All brains are different, and not every person with PPA will fit perfectly into a specific category. However, there are some general trends that tend to be present. 

1 Semantic (PPA-S)

This is a problem of word understanding. The person’s speech is often fluent, but it can be filled with “empty” words–for example, “the stuff there.” In addition, the person may easily forget familiar words or fail to understand common words. An interaction with a person with PPA-S may sound like this: 

Partner: “Can you hand me the spoon?”

Person with PPA: “Spoon? What is a spoon?” (They will recognize it when it’s placed in their hand.)

2 Agrammatic (PPA-A/PPA-G)

This is a problem of word order and word production. The person’s speech is usually nonfluent, effortful, and shorter in length. Word order will be unusual, with word substitutions. For example, the person may say  “no” for “yes,” or “he” for “she.”

The person understands simple words and phrases, but their comprehension declines with longer and more complex sentences. 

3 Logopenic (PPA-L)

This is a problem of word finding. The person’s speech is generally fluent, but it often contains “empty” words–for example, “whatchamacallit,” “thingamajig,” or “you know that thing.” The person will have an increased difficulty with repeating words and sentences. 

How is PPA diagnosed?

Typically, a neurologist will conduct language tests and brain imaging tests. A neuropsychologist may conduct additional assessments and track language decline for a period of two years. 

Once a person is diagnosed with PPA, a speech-language pathologist can do an in-depth language and cognitive assessment. These tests will help determine the person’s current language function and communication needs.  

How is primary progressive aphasia treated?

It’s important to factor in the progressive nature of a PPA diagnosis. Speech and language therapy should be started as soon as possible so a care plan can be created. The speech therapist can identify strategies that will maximize the person’s communication and improve their quality of life. 

Every person with PPA is different, so specific treatment programs may vary as well. In addition, the course of treatment may change as the condition affects more areas of language and cognition. 

With the progression of PPA, speech therapy may focus on compensatory strategies to help the person communicate.

In PPA’s least severe and early stages, speech therapy may include treatments that focus on improving language impairments. Some examples are Verb Network Strengthening Treatment, Semantic Feature Analysis, and Copy and Recall Treatment.

With the progression of PPA, speech therapy may focus on compensatory strategies to help the person communicate. These compensatory strategies can range from low-tech, such as a communication card in the person’s wallet, to high-tech, such as voice banking or augmentative communication devices. These strategies may also include environmental changes, like making frequently requested items more accessible to the person.

The importance of involving communication partners

Having a language impairment can make it hard for a person to interact independently in the community. That’s why speech therapy is not limited to the person with PPA alone. Treatment should also include the person’s communication partners. Programs such as Supported Conversation in Aphasia (SCA) and Promoting Aphasic’s Communication Efficiency (PACE) are essential training for friends and family who often communicate with a person with PPA. These programs teach people techniques they can use to support and promote communication.

Because the person with PPA will be faced with changes in language function, they will need to learn new ways to communicate as time goes on. Speech therapy can provide ongoing education and support to help the person with PPA stay connected to others.

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