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Genetic and Rare Diseases Information Center (GARD)

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Primary progressive aphasia

Other Names for this Disease
  • Aphasia, primary progressive
  • PPA
  • Primary progressive aphasia syndrome
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Do you have any information on the stages of progressive primary aphasia (PPA) semantic variant?

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What are the stages of primary progressive aphasia semantic variant?

Symptoms of the semantic variant of primary progressive aphasia typically begin between the ages of 55 and 70.  Half of affected individuals were living 13 years after symptoms began.  The first sign of this condition is when an individual has persistent difficulty finding the right word when speaking.[1]  Speech, grammar, and the ability to understand sentences usually remain normal in the initial stage.  As the disease progresses, affected individuals leave out an increasing number of words from the sentences they speak and substitute general words for particular words (for example, they say "animal" instead of "cat").  Eventually, individuals with the semantic variant may develop other features of this disease, such as depression, eating disorders, or changes in social behavior.[1][2]

Additionally, one article studied the progression of primary progressive aphasia in general (that is, they did not distinguish between the variants).  This article found that the average age when affected individuals first developed symptoms is 62.  The time frame until total language loss varied between 5 to 10 years.  Daily living activities first became more difficult 6 to 7 years after the first symptoms appeared; affected individuals were unable to care for themselves for the last 1 to 2 years of life.  Overall, individuals with primary progressive aphasia lived an average of 7 years after symptoms first developed.[3]
Last updated: 2/20/2013