Primary Progressive Aphasia: Symptoms, Types, Treatment and Prognosis

Primary progressive aphasia, or PPA, is a type of frontotemporal dementia (FTD) that affects speech and language. The word "aphasia" refers to difficulty with expressive and/or receptive communication. This neurological syndrome causes people to slowly lose their ability to read, write, speak, and understand language.

Unlike Alzheimer's disease (AD), other cognitive functions tend to remain intact in early PPA. PPA is not the same as AD, although AD is thought to cause PPA in 30% to 40% of cases.

Symptoms of PPA

Initial symptoms of PPA include difficulty recalling a specific word, substituting a closely related word, such as "take" for "tack," and comprehension problems. People with PPA can often perform intricate tasks but have difficulty with speech or language. For example, they may be able to build a complicated house but not be able to express themselves well verbally or understand what others are trying to communicate to them.

As the disease progresses, speaking and understanding written or spoken words becomes more difficult, and many people with PPA eventually become mute. On average, a few years after these initial symptoms involving language appear, PPA begins to affect memory and other cognitive functions, as well as behavior.

Who Gets PPA?

PPA is classified as a rare disease, however, many may be undiagnosed since they might not seek medical help or be misdiagnosed due to the unfamiliarity with PPA.

Interestingly, men develop PPA about twice as often as women. The average age of onset is between 50 and 70. Those who get PPA are more likely to have a relative with some type of neurological problem.

Causes of PPA

PPA happens when tissues in the frontal, parietal, and temporal regions (lobes) of the brain gradually deteriorate over time, usually on the left side of the brain. This is where several speech, language, and memory "control centers" are located.

Underlying neurodegenerative diseases causing PPA. The most common are:

• Frontotemporal lobar degeneration with abnormal tau protein accumulation (FTLD-tau)
• Frontotemporal lobar degeneration with abnormal TDP-43 accumulation (FTLD-TDP-43)
• Alzheimer’s disease

In nearly all cases PPA is not genetic however, very seldom it can be hereditary. The most common gene implicated in familial PPA is the progranulin gene (GRN).

Categories of PPA

PPA can be subdivided into three categories:

• Semantic PPA: Individuals lose the ability to say certain words, and their ability to recognize other words may decline.
• Nonfluent/Agrammatic PPA: Individuals have difficulty forming complete sentences. For example, they may be able to speak using nouns and verbs, but not be able to connect them with words like "to" and "from." As agrammatic PPA progresses, individuals may struggle with forming any words and may have trouble with swallowing and muscle control.
• Logopenic PPA: Individuals may experience difficulty locating the correct words to speak but retain the ability to understand what others are saying to them. Comprehension is relatively well preserved, but not completely.

Treatment

There is no drug specifically approved to treat PPA. Management of the disease includes attempting to compensate for the language difficulties by using computers or iPads, as well as a communication notebook, gestures, and drawing. Cards pre-printed with certain phrases or words may also help allow the person to express himself. Other approaches involve training on word retrieval by a speech therapist.

Additionally, some research that involved providing language activities, communication techniques, counseling, and education to people living with PPA and their spouses demonstrated a significant improvement in communication and coping at its completion.

Additional support unrelated to language ability can be helpful as well. Difficulties with sleep, anxiety, and mood are common. Certain medications, like selective serotonin reuptake inhibitors (SSRIs) can help to manage the anxiety, depression, and changes in behavior associated with PPA.

Good nutrition (fruits, green leafy and other vegetables, whole grains, fish, poultry, and healthy fats including nuts) should also be a goal. Try to limit poor food choices, skipped/forgotten meals, and unhealthy weight gain or loss.

Prognosis and Life Expectancy

While some people with PPA can continue working for quite some time, others find that they are unable to perform at their job, especially if their work requires a higher level of communication and collaboration with others. Language ability slowly declines over years. 

PPA eventually progresses to more generalized/common forms of the person's underlying neurological condition (Alzheimer's, FTD). Because of this, changes in memory, attention, or personality develop, and movement or swallowing may become affected.

As with other frontotemporal dementias, the long-term prognosis is limited. The typical life expectancy from the onset of the disease is 3 to 12 years. Often, complications from PPA, such as swallowing difficulties, lead to the eventual decline.

A Word From Verywell

We at Verywell understand that primary progressive aphasia can be a difficult diagnosis to receive, both as an individual and as a family member of someone with PPA. Most people benefit from connecting with others in similar situations as they cope with the challenges that develop from PPA. One resource available nationwide is the Association for Frontotemporal Dementia. They offer several local support groups, as well as online information and phone support.