Primary Progressive Aphasia: Symptoms, Types, Treatment and Prognosis

Primary progressive aphasia, or PPA, is a type of frontotemporal dementia that affects speech and language—thus, the word "aphasia" which refers to difficulty with expressive and/or receptive communication. Unlike Alzheimer's disease, cognitive functioning remains intact in early 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 become more difficult, and many people with PPA eventually become mute.

On average, about five years after these initial symptoms involving language appear, PPA begins to affect memory and other cognitive functions, as well as behavior.

PPA is classified as a rare disease, and it's estimated that fewer than 200,000 people in the United States are living with this disorder. However, many may be undiagnosed since they might not seek medical help or be misdiaganosed due to the unfamiliarity with PPA. Interestingly, about twice as many men than women develop PPA. The average age of onset is 60 years old, with most cases ranging from age 40 to 80. Those who get PPA are more likely to have a relative with some type of neurological problem.

People who develop PPA show atrophy in the area of the brain where speech and language are controlled. Some cases of PPA have a genetic component found in a mutation of the GRN gene.

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.
• 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.

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 be helpful in allowing 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 persons living with PPA and their spouses demonstrated a significant improvement in communication and coping at its completion.

While some people with PPA are able to 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.

As with other frontotemporal dementias, the long-term prognosis is limited. The average life expectancy from onset of the disease is 8 to 10 years. Often, complications from PPA, such as swallowing difficulties, often lead to the eventual decline.

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.