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Primary Progressive Aphasia

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Updated May 06, 2014

Written or reviewed by a board-certified physician. See About.com's Medical Review Board.

Primary progressive aphasia, or PPA, is a type of frontotemporal dementia that affects speech and language. Unlike Alzheimer's disease, cognitive functioning remains intact in early PPA.

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 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.

Who Gets PPA?

Interestingly, about twice as many men than women experience PPA. The average age of onset is 60 years old, with most cases ranging from age 40 to 80. There is not a definitive genetic link, although those who get PPA are more likely to have a relative with some type of neurodegenerative problem.

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.

  • 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.

Treatment of PPA

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. Other approaches involve training on word retrieval by a speech therapist.

Prognosis

As with other frontotemporal dementias, the 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, lead to the eventual decline.

Sources:

The Association for Frontotemporal Degeneration. http://www.theaftd.org/

National Aphasia Association. Diagnosing Primary Progressive Aphasia. Accessed January 27, 2012. Diagnosing Primary Progressive Aphasia

National Center for Biotechnology Information. U.S. National Library of Medicine. Pub Med Health. Pick’s Disease. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001752/

National Institutes of Health. Office of Rare Diseases Research. Primary Progressive Aphasia. Accessed January 27, 2012. http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=8541

Northwestern University. Feinberg School of Medicine. Primary Progressive Aphasia. Accessed December 11, 2011. http://www.brain.northwestern.edu/ppa/

Northwestern University. Feinberg School of Medicine. Treatment. Accessed January 27, 2012. http://www.brain.northwestern.edu/ppa/treatment.html

University of California, San Francisco. Forms of Frontotemporal Dementia. Accessed December 11, 2011. http://memory.ucsf.edu/ftd/overview/ftd/forms/multiple

U.S. National Institutes of Health. National Institute on Aging. Types of Frontotemporal Disorders. Accessed December 12, 2011. http://www.nia.nih.gov/Alzheimers/Publications/FTLD/02.htm

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