Primary Progressive Aphasia

Primary progressive aphasia (PPA) is a condition that slowly damages the parts of the brain that control speech and language. People with PPA usually have difficulty speaking, naming objects or understanding conversations.

What Causes PPA?

The clinical symptoms of PPA are caused by degeneration in the parts of the brain that control speech and language (typically the left side of the brain in the frontal, temporal and parietal regions).

What Happens in PPA?

This type of aphasia begins gradually, with speech or language symptoms that vary depending on the brain areas affected by the disease. For example, in one type of PPA, people may initially have trouble producing speech, whereas, in another variant, word-finding and comprehension problems are more pronounced.

PPA has three variants:

1. Semantic (svPPA): people with svPPA experience increasing trouble naming people, objects, facts and words
2. Nonfluent/agrammatic (nfvPPA): people with nfvPPA tend to come to the doctor's office with complaints of difficulty pronouncing words
3. Logopenic (lvPPA): word-finding difficulties are the most prominent feature in patients with logopenic PPA

These syndromes result from a variety of underlying diseases, but frontotemporal lobar degeneration (FTLD) (both tau and TDP-43 subtypes) or Alzheimer’s disease is most often the cause.

The majority of people with PPA have problems expressing themselves with language, while their memory stays relatively intact, especially during the first two years of decline. Difficulties reading and writing may develop as the disease progresses. At the UCSF Memory and Aging Center, we have found a small group of patients with PPA who develop new creative skills in music and art as their language skills decline.

Are There Medicines to Treat PPA?

People with language difficulties may benefit from speech therapy to help them learn alternative ways to supplement and compensate for their lost skills. Maintaining adequate communication and social connections are critical. Unlike many people who develop aphasia from head injury or stroke, people with PPA do not typically improve with time, but a therapist may be helpful in maximizing abilities and exploring other ways to communicate. Non-verbal techniques for communicating, such as gesturing or pointing to pictures may help people express themselves.

Aphasia identification cards explaining that the person has a language problem may be helpful. Many speech pathologists and occupational therapists have their own practices, while others are available through local hospitals and medical centers. Ask your doctor for a referral.