Primary Progressive Aphasia

What is Primary Progressive Aphasia (PPA)?

Primary Progressive Aphasia (PPA) is a neurological syndrome in which language capabilities become slowly and progressively impaired. Unlike other forms of aphasia that result from stroke or brain injury, PPA is caused by neurodegenerative diseases, such as Alzheimer’s Disease or Frontotemporal Lobar Degeneration. PPA results from deterioration of brain tissue important for speech and language. Although the first symptoms are problems with speech and language, other problems associated with the underlying disease, such as memory loss, often occur later.

PPA commonly begins as a subtle disorder of language, progressing to a nearly total inability to speak, in its most severe stage. The type or pattern of the language deficit may differ from patient to patient. The initial language disturbance may be fluent aphasia (i.e., the person may have normal or even increased rate of word production) or non-fluent aphasia (speech becomes effortful and the person produces fewer words). A less common variety begins with impaired word-finding and progressive deterioration of naming and comprehension, with relatively preserved articulation.

As with aphasia that results from stroke or brain trauma, the manifestations of PPA depend on what parts of the left hemisphere are relatively more damaged at any given point in the illness. The person may or may not have difficulty understanding speech. Eventually, almost all patients become mute and unable to understand spoken or written language, even if their behavior seems otherwise normal.

Signs and symptoms of other clinical syndromes are not found through tests used to determine the presence of other conditions. PPA is not Alzheimer’s disease. Most people with PPA maintain ability to take care of themselves, pursue hobbies, and, in some instances, remain employed.

Is there any treatment or assistance for people with PPA?

People with primary progressive aphasia are fighting against a condition in which they will continue to lose their ability to speak, read, write, and/or understand what they hear. Usually people with aphasia that results from stroke or head injury will experience improvement over time, often aided by speech therapy. This is not the case for people with primary progressive aphasia. However, individuals with PPA may benefit during the course of their illness by acquiring new communication strategies from speech-language pathologists. Some families have also learned new strategies through participation in Aphasia Community Groups.

Many people with aphasia find it helpful to carry identification cards and other materials that can help explain the person’s condition to others. ID cards are available from the the National Aphasia Association website. Some communication-assistive devices may also be helpful. Non-verbal techniques for communicating, such as gesturing and pointing to pictures, may help people with PPA express themselves.

En Español, Afasia primaria progresiva