Primary Progressive Aphasia

What is Primary Progressive Aphasia?

Aphasia is a problem in the brain that effects how a person talks, reads, writes, recognizes others and interacts with the world. In Primary Progressive Aphasia or PPA the parts of the brain responsible for speech and language have become damaged or have deteriorated.

Although the patient may gradually find difficulties reading, writing or talking, the patient’s intelligence is still intact. This condition gets worse over time until the person can no longer talk. The most common cause is a neurodegenerative disease such as frontotemporal lobar degeneration and Alzheimer’s disease. Just why people get neurodegenerative diseases are unknown. There is no cure. Patients can live years after diagnosis.

What are the Symptoms of Primary Progressive Aphasia?

There are three main types of PPA: sematic variant PPA, nonfluent or agrammatic PPA, and logopenic variant PPA. They exhibit different symptoms.

Sematic variant PPA patients cannot remember the names of things. They may even say “things” or similar word to substitute for all the names they have forgotten. Eventually they may not understand many words spoken to them. Sometimes they also can no longer recognize faces.

Nonfluent or agrammatic variant PPA patients have trouble pronouncing words and trouble following long sentences. Eventually they lose the power to talk and often show symptoms similar to that of Parkinson’s disease.

Logopenic variant PPA patients also suffer from Alzheimer’s disease. The left posterior temporal cortex part of the brain shrinks or generates. Patients may still understand grammar or what is being told to them for years but talking becomes increasingly difficult and punctuated with long pauses between words. They cannot follow a long conversation or movie.

Primary Progressive Aphasia Causes

Scientists do not fully understand the causes of Primary Progressive Aphasia. They do know that it is a “degenerative” disorder, which is to say, it causes gradual and progressive cell death in the brain, rather than damage as a result of a shock or trauma. In Primary Progressive Aphasia, this degeneration is most pronounced in the parts of the brain associated with language production, which causes the difficulties people experience generating speech.

Primary Progressive Aphasia can develop in patients with similar degenerative mental conditions. Alzheimer’s Disease is one (30%-40% of cases), but it is much more common in patients who suffer from a condition called Frontal Lobe Degeneration (60-70% of cases).

In around 38%-50% of cases, Frontal Lobe Degeneration is hereditary. This has led scientists to believe there may be genetic factors involved in the development of Primary Progressive Aphasia.

In fact, researchers have identified a mutation in a specific gene that may be at the root of Frontal Lobe Degeneration. This mutation prevents the production of a protein called progranulin, which appears to have a protective and anti-inflammatory effect on the brain. Researchers believe low progranulin levels may be a cause of Primary Progressive Aphasia, but more work needs to be done before they know for sure.

How is Primary Progressive Aphasia Treated?

There is no known cure or even effective treatment for PPA. Patients must learn to express themselves in other ways such as through drawing or in music, since language based on words has now been taken from them.

Patients are advised to always carry medical identification bracelets and cards letting first responders know that in case of emergency that the patient suffers from PPA. Patients and their loved ones often learn a new language consisting of gestures and pantomime. Working with a speech therapist may help patients, their caretakers and their loved ones come up with such a language.

Primary Progressive Aphasia Prevention

There is no proven measure that can be taken to prevent Primary Progressive Aphasia. As noted earlier, scientists have identified lower levels of the protein progranulin in people who suffer from Primary Progressive Aphasia. Some researchers have speculated that the replacement of this protein may prevent the frontal lobe degeneration that leads to Primary Progressive Aphasia.

Prevention could involve screening for low levels of progranulin in people at risk of developing degenerative mental disorders, and then providing some form of replacement therapy. However, although this is a promising line of inquiry, this research area is in its early phases and no clinical trials have been conducted.

Last Reviewed:
October 09, 2016
Last Updated:
January 11, 2018
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