What is Primary Progressive Aphasia?

UofSC researcher offers explanation of rare disorder

Aphasia is a medical condition that affects a person’s ability to communicate. People experiencing it may have trouble speaking, writing or understanding language.

Unlike other forms of the condition, primary progressive aphasia is neurodegenerative.

“In most cases, aphasia is caused by stroke-related damage to the brain,” says Julius Fridriksson, SmartState Endowed Chair at the Arnold School of Public Health and director of the Aphasia Lab at the University of South Carolina. “PPA, which is a relatively rare disorder, differs from stroke aphasia in that it is caused by gradual atrophy selectively affecting language regions of the brain. It is important to note that in both cases – stroke aphasia and PPA – the problem has to do with language processing and not intelligence.”

There are three variants of primary progressive aphasia, depending on which portion of the brain is affected: semantic, which primarily manifests as problems with language comprehension; logopenic, which causes conversational pauses and difficulty finding words; and nonfluent-agrammatic, a variety that affects grammatical skills and the fluency of speech.

Currently, there is no cure. For patients with general aphasia, language skills can often be recovered, either on their own or with therapy. But with primary progressive aphasia, the disorder cannot be reversed, though treatment can help patients manage their symptoms or slow their progression.

While primary progressive aphasia patients do eventually lose the ability to speak, write and understand language, the disorder is not a death sentence. Treatment may help them learn alternative strategies for communication, and many continue to lead otherwise independent and active lives. Furthermore, new research into treatments is promising.

Recent advances suggest cholinesterase inhibitors, a class of drugs typically administered for Alzheimer’s disease, may also benefit people with PPA,” Fridriksson says. “Other studies are also coming online suggesting that behavioral speech therapy may aid in preserving language function in PPA. Hopefully, a combination of both drug and behavioral aphasia therapy will bring about some real relief here sometime soon.”

Sources: The Mayo Clinic, The National Aphasia Association, Julius Fridriksson

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