What is primary progressive aphasia?

Primary progressive aphasia, or PPA, is a rare neurological syndrome that gradually destroys language. Northwestern University's Mesulam Center says that unlike other dementias, its earliest symptoms are trouble speaking, finding words and understanding language rather than memory loss. Adults of any age can develop it, though it is most often diagnosed before 65. Communication becomes harder as it progresses.

Yes. The National Institute on Deafness and Other Communication Disorders describes PPA as dementia in which aphasia is the first and most noticeable symptom, rather than memory, behavior or movement changes. PPA worsens over time until the person may lose the ability to use language, and other thinking, behavioral and movement symptoms emerge as the disease progresses.

PPA happens when harmful proteins build up in the parts of the brain that control language, gradually damaging and killing brain cells. Northwestern University's Mesulam Center says most cases are caused by frontotemporal lobar degeneration, and Alzheimer's disease causes the rest. Diagnosis involves ruling out other conditions, since there is no single definitive test, using history, exams, lab tests and imaging.

There are three subtypes. Northwestern University's Mesulam Center describes agrammatic or nonfluent PPA as effortful speech with shorter sentences and missing words; semantic PPA as losing the meaning of single words and names of familiar objects; and logopenic PPA as trouble finding words in conversation, often substituting a different word. Symptoms vary by subtype, but all involve progressive language loss.

There is no cure, so treatment aims to keep language skills and add new ways to communicate. The National Institute on Aging suggests a communication notebook of labeled photos, gestures and drawings, word lists stored on a phone, and speaking slowly in simple sentences. It advises working with a speech-language pathologist familiar with PPA, since stroke-related aphasia needs different strategies.