Limited research suggests that those with primary progressive aphasia (PPA) live on average from 7 to 12 years after symptoms onset. However, PPA does not appear to be a direct cause of death.

Primary progressive aphasia (PPA) is a rare neurological disorder that involves the gradual deterioration of language skills. It’s caused by atrophy to parts of the brain that deal with speech (the frontal or temporal lobes) and is linked to Alzheimer’s disease and frontotemporal dementia.

Over time, those with this condition may lose their ability to speak, write, or understand spoken or written language.

There’s currently no known cure for aphasia, although speech therapy, medication, and other treatments may help slow the progression of symptoms.

PPA survival rates vary depending on the type you’re diagnosed with, and they’re based on extremely limited research. Current estimates vary between 7 to 12 years after symptoms initially appear.

Keep in mind that the leading causes of death of patients with PPA include cardiac arrest and pneumonia, not the disorder itself. Although PPA may make day-to-day life challenging, it does not directly lead to one’s death.

Here’s what else to know about the outlook for this condition.

According to limited 2021 research of 83 deceased patients, the average survival rates for the three most common variants of PPA were as follows:

  • Logopenic variant primary progressive aphasia (lvPPA): This type involves issues recalling words. In the study, patients lived on average 7.6 years after symptoms appeared.
  • Nonfluent variant primary progressive aphasia (nfvPPA): This type involves issues with articulation, pronunciation, or vocalizing. Patients with this type lived an average of 7.1 yearsafter symptoms appeared.
  • Semantic variant (svPPA): This type involves issues in understanding the meaning of words. svPPA is also associated with preserved fluency, repetition, and grammar. Those with this variety lived an average of 12 years post-symptoms onset.

Since the disease is rare and the sample size is small, however, researchers admit that it’s challenging to draw any conclusions about this data. They also note that one’s region and age at diagnosis could also influence patients’ survival rate.

PPA typically appears between the ages of 50 and 65, an age range when other comorbid conditions, such as heart disease, may be developing as well.

There’s no research to suggest that PPA directly causes death.

According to limited 2021 research of just 34 patients, the leading causes of death for those with PPA were:

Other major cardiovascular events, such as strokes, also were the cause of 12% of the 34 patients’ deaths.

Those with the nfvPPA variant of the condition often have aspiration pneumonia in particular.

PPA is also linked to Alzheimer’s and frontotemporal dementia, which present inherent challenges to day-to-day life and may contribute to shortening one’s lifespan.

The stages of PPA progression range from mild to severe and include:

  • Very mild: During this time, loss of language or communication skills may be very subtle and attributed to aging or stress.
  • Mild: In this stage, communication issues are generally apparent to both the individual as well as others.
  • Moderate: As the condition advances, the individual may need support dealing with day-to-day tasks and will typically have to stop working, if applicable. This typically happens after 1–2 years after symptoms appear, but it may vary based on the type of PPA.
  • Severe: At this stage, many daily tasks may become increasingly challenging. The individual also may not be able to live alone anymore.
  • Very severe: As the condition progresses, meaningful communication as well as mobility may be drastically affected. The person may need assistance grooming, using the restroom, etc.
  • Profound: At this stage, communication may become impossible, and the person may become essentially immobile.

How quickly does PPA progress?

PPA progression may vary widely. It tends to develop gradually and worsen over the course of several years, ranging anywhere from 2 to over 10 years.

Symptom progression also may depend on the variation of the disease. For instance, the svPPA type appeared to take the longest to diagnose and seems to be the slowest to progress, often unfolding over the course of at least 10 years.

Other types of PPA may progress much more rapidly. However, since the disease is rare and sample sizes remain very small, more research is necessary before drawing conclusions.

Even though there’s currently no cure for PPA, there are ways to slow its progression and improve one’s day-to-day quality of life. To help manage symptoms and improve communication, experts typically recommend the following treatments:

  • Speech and language therapy: Research shows that speech and language therapy that includes tasks like vocabulary tests and verbal fluency can benefit those with PPA.
  • Communication strategies: Alternative communication, like using gestures or alternative communication devices (e.g., tablets, visual aids), can help those with PPA communicate more effectively.
  • Transcranial direct current stimulation (tDCS): A 2021 review found that tDCS, which involves applying a mild electrical current to the scalp to stimulate brain activity, may improve language skills. Still, more research is needed to know for sure.
  • Medication: 2022 research suggests that medication use for treating PPA is limited. Still, scientists think that anti-depressants, anti-anxiety medications, cholinesterase inhibitors (ChEIs), sleep aids, antipsychotics, or N-Methyl-D-aspartate receptor antagonists (NMDA antagonists) may help manage some PPA-related symptoms.

Though PPA doesn’t directly cause death, research suggests that the survival timeline after onset is anywhere from 7 to 12 years.

PPA typically manifests later in life, when comorbid conditions, like heart problems, are also more likely. In a small sample, cardiac arrest and pneumonia were the leading causes of death in those with PPA.

Though PPA can make day-to-day tasks and communication increasingly difficult, there are ways to manage and slow its progression. Treatments like speech therapy can help patients improve their communication skills and quality of life, especially early in the disease process.