Primary progressive aphasia

Signs and symptoms of primary progressive aphasia (PPA) usually begin gradually and vary based on the type of PPA present. In addition to speech problems, reading and writing also are usually affected. Symptoms generally worsen over time. Affected people may become mute and eventually lose the ability to understand written or spoken language.^[3] Although additional cognitive symptoms may emerge later in the course of the illness, deficits must be generally confined to the area of language for at least a couple of years to fulfill a diagnosis of PPA.^[4]

People with the semantic dementia type may have difficulty with:^[3]

• understanding spoken or written language, particularly single words
• understanding word meanings
• naming objects

People with the lopogenic type may have:

• difficulty retrieving words in speech
• frequent pauses in speech while searching for words
• slow speech
• difficulty repeating phrases or sentences

People with the progressive non-fluent type may have:

• difficulty speaking
• hesitant, halting speech
• errors in speech sounds
• difficulty understanding sentences
• difficulty using grammar correctly

Some people with PPA are ultimately diagnosed with other neurodegenerative diseases.^[5]

There is limited information in the medical literature about the long-term outlook (prognosis) for people with primary progressive aphasia (PPA) and what ultimately causes death in affected people. PPA may be the form of onset of any of a number of different neurodegenerative diseases.^[5] Prognosis may therefore vary depending on whether another neurodegenerative disease develops, and what that disease is.

While the age of onset of PPA tends to fall between the ages of 50 and 70 years, some people may become affected before or after this age range. The condition tends to progress, developing into mutism in many cases. Loss of independence occurs at a later stage than in other types of dementia (at 7 years in 50% of all affected people, according to one author). Survival typically ranges from about 7 to 10 years, although recent studies suggest that the course of PPA may be more gradual.  In some people, language deficiency remains the predominant symptom, or even the only one. Others may develop additional problems including cognitive, behavioral, or extrapyramidal deficits (such as Parkinsonism). It is not known how often people with PPA develop generalized dementia or additional deficiencies (which is called ‘PPA plus’) in the course of this disease. In one follow-up study by Kertesz et al., 54% of the people in the study developed a second or third syndrome other than PPA (such as frontotemporal dementia, progressive supranuclear palsy, or corticobasal degeneration) during monitoring.^[5]  Alzheimer disease (AD) is found in many autopsies, but it is not known whether AD can cause PPA in its early stages or if two neurodegenerative diseases are coexisting as a result of shared causes.^[5]

Because various neurodegenerative diseases may ultimately be diagnosed in people with PPA, it is not possible to predict the timing and nature of future complications in affected people. If another neurodegenerative disease is diagnosed, the prognosis (including possible future causes of death) is likely to relate to the specific disease present. However, one of the most common causes of death for many associated conditions including progressive supranuclear palsy and frontotemporal dementia is pneumonia.^[6][7]

People with questions about the specific prognosis for themselves or family members should speak with their health care provider.