Primary Progressive Aphasia: Causes, Types, and Symptoms

Primary progressive aphasia is a neurological syndrome that slowly and progressively impairs a person’s language abilities.

Symptoms usually begin gradually, often starting with minor problems with naming. As symptoms worsen, patients eventually may lose the ability to understand written or spoken language.

In this article, you will learn the major causes of primary progressive aphasia, and how to recognize its early signs. Although there is currently no treatment, early intervention with speech therapy can sometimes slow the progression and help patients maintain their independence for as long as possible.

Use the links below to jump straight to any section:

• Causes
• Types and symptoms
• Complications
• Speech therapy
• Management tips

Causes of Primary Progressive Aphasia

Unlike other types of aphasia, primary progressive aphasia (PPA) is not caused by direct brain damage from a stroke or brain injury. Rather, it is a type of frontotemporal dementia, a group of disorders that lead to the degeneration of the frontal or temporal lobes of the brain.

With primary progressive aphasia, the tissue of the language center of the brain slowly deteriorates, causing a gradual loss of language skills. While it is not directly caused by stroke or brain injury, sometimes the conditions occur together.

The shrinking of these brain regions is due to the presence of abnormal proteins common in Alzheimer’s and other forms of dementia. Risk factors for primary progressive aphasia include:

• Genetic mutations. Certain genetic mutations are linked to this type of aphasia. If a family member has PPA, you are more likely to develop it yourself.
• Learning disability. Being diagnosed with a learning disability as a child, particularly developmental dyslexia, increases your risk of primary progressive aphasia. However, most people who have dyslexia will not develop PPA.

If you have any of the risk factors associated with progressive aphasia, it is important to understand the early signs of this disorder so that you can seek immediate treatment.

Types and Symptoms of Primary Progressive Aphasia

Symptoms of primary progressive aphasia typically begin between the ages of 50-70 years. Early symptoms can vary, depending on which part of the brain’s language areas deteriorate first.

In fact, there are three main types of progressive aphasia that a person can develop, each causing different initial symptoms. We will examine each type in the sections below:

1. Logopenic Variant  

This type first appears most similar to anomic aphasia. Initial symptoms include:

• Difficulty retrieving words
• Frequent pauses while searching for words
• Difficulty repeating phrases

2. Semantic Variant

This type is similar to fluent aphasia in that it typically affects language comprehension first. Signs and symptoms can include:

• Difficulty understanding spoken or written language
• Trouble with word definitions
• Struggling with naming objects

3. Nonfluent-agrammatic Variant

Finally, this type shares many characteristics with expressive aphasia, which affects a person’s ability to produce words. Symptoms may include:

• Slow, laborious speech
• Difficulty understanding complex sentences
• Poor grammar when writing or speaking
• Sometimes accompanied by speaking errors such as incorrect speech sounds (also known as apraxia of speech)

Because PPA is a progressive disease, symptoms will usually gradually worsen with time.

Complications of Primary Progressive Aphasia

Although each type of primary progressive aphasia begins with different initial symptoms, all three eventually lead to a complete loss of language skills.

As the condition progresses, other cognitive functions, such as memory and concentration, can become impaired. Some people also develop motor disorders such as difficulty with movement and coordination. This can lead them to require help with daily care.

Finally, many patients with PPA can develop depression as a result of the social isolation that the disease causes. Other problems can include poor decision-making and inappropriate behavior. These occur as a result of further deterioration of the frontal lobe.

Speech Therapy for Primary Progressive Aphasia

Although there is no treatment for primary progressive aphasia, speech therapy is sometimes able to slow the progression of the disease and maintain function.

Speech therapy exercises work by activating neuroplasticity. Neuroplasticity refers to the brain’s ability to form neural pathways in response to repetition or changes in environment. These pathways can allow undamaged portions of the brain to take control of functions that were previously controlled by damaged ones.

Therefore, even if progressive aphasia damages the brain regions that normally control language production, it may still be possible for other areas to compensate.

To activate neuroplasticity, the patient must engage in repetitious exercise. The more you stimulate your brain through exercise, the more neural pathways your brain will create in response. With these new neural pathways in place, you can begin to regain speech function.

One helpful way to practice therapy exercises every day is to use speech therapy apps, such as the CT app. This top-rated app was designed by speech-language pathologists to help patients regain speech, memory, and cognitive function. Featuring over 100,000 exercises that you can tailor to your individual needs, it can help slow the progression of your aphasia and possibly even restore some of your function.

Managing Progressive Aphasia

There is no cure for progressive aphasia. Instead, treatment is focused on slowing the progression of the disease. Speech therapists also seek to help the person adapt to loss of language skills. For example, some patients benefit from learning sign language or other alternative communication techniques.

Some ways to manage primary progressive aphasia that a speech therapist might teach you and your family members include:

• Ask yes or no questions. Family members and friends should try to use yes or no questions as often as possible. This puts less strain on the person with aphasia and will make conversation flow smoother between you two.
• Listen. The best way to slow the progression aphasia is to practice speaking. Therefore, let them speak as much as possible, even if it is difficult. If you feel like they really need help, ask first before assisting them.
• Stick to small groups.A small group puts less pressure on the person with aphasia to do all the talking, and allows them to respond when they feel like it. Be sure to include your loved one in conversation and keep them involved, but don’t push them too hard.
• Use pictures and drawings. The left side of the brain controls language, but the right is responsible for non-verbal and visual functions. This means the ability to draw and interpret images is usually still intact in people with progressive aphasia. If communication is especially difficult, try drawing what you mean.

Electronic devices might also be able to assist or replace speech in some people. However, because they require a person to understand conventional language, they are more suited for patients whose cognitive skills are not yet severely impaired.

Understanding Primary Progressive Aphasia

Primary progressive aphasia is a neurological disease that causes a person to gradually lose their language skills.

The disease progresses slowly, and it can take several years before the patient completely loses their ability to communicate. Other complications associated with PPA include depression, impulsivity, and difficulty coordinating movements.

Speech therapy can help slow the progression of the aphasia and preserve the patient’s communication skills for a few more years. Speech therapists can also teach alternative  techniques that can allow patients and their families to communicate even when traditional speech is no longer possible.

We hope this article gives you a better understanding of how to live with and manage primary progressive aphasia. Talk to a specialist for more detailed information on specific treatments and coping methods.