Primary progressive aphasia (PPA) is a neurological condition that affects a person’s ability to communicate. PPA has three subtypes. Damage due to frontotemporal dementia or Alzheimer’s disease is likely to cause PPA.

Other forms of aphasia may result from brain injury or stroke. The symptoms of PPA depend on the location of damage in the brain, but generally, people may have trouble expressing their thoughts, understanding, or finding words.

The condition can affect different aspects of language, such as grammar, vocabulary, and pronunciation. In some cases, people with PPA may experience changes in their behavior, such as becoming more impulsive or socially withdrawn.

There is currently no cure for PPA, but speech therapy and other communication strategies can help manage the symptoms and improve a person’s quality of life.

This article examines PPA, including its causes, symptoms, and treatments.

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PPA can cause a gradual loss of language abilities, usually without significant memory impairment. Degeneration of tissue in the brain, usually due to a neurodegenerative disease such as Alzheimer’s disease or frontotemporal dementia, can cause PPA.

Symptoms may start when someone is between the ages of 50 and 70 years. The condition can also affect people outside that age range, but this is rare.

Doctors divide PPA into three subtypes, which cause distinct language difficulties:

  • Semantic variant PPA: People have trouble understanding the meaning of words and recognizing objects or faces.
  • Logopenic variant PPA: People have trouble finding the right words to express themselves and producing grammatically correct sentences.
  • Nonfluent/agrammatic variant PPA: Individuals with this subtype find it more and more difficult to speak but can still recall what individual words mean.

PPA is a progressive condition. In the early stage, people may have symptoms such as slowed speech, difficulty finding words, and problems with reading and writing. They may also need help to follow complex conversations or express themselves clearly.

As the disease progresses, it may affect other cognitive abilities, and the person may have difficulty with movement and swallowing. Also, the person may begin to rely on nonverbal communication to express their needs and wants. However, symptoms largely depend on which area of the brain the condition affects.

Certain risk factors, such as family history of PPA, can increase a person’s likelihood of developing this condition.

The symptoms of PPA depend on the subtype and the part of the brain in which degeneration is occurring.

Symptoms of semantic variant PPA include difficulties with:

  • understanding the meaning of words
  • recognizing familiar people, objects, or places
  • naming objects or people
  • understanding spoken or written language
  • reading and writing words with unusual pronunciation or spelling rules

Symptoms of logopenic variant PPA include:

  • repetition of words or sentences
  • difficulty with grammar and sentence structure
  • trouble understanding complex sentences
  • trouble finding the right words to express oneself
  • difficulty with speech sounds, such as substituting or omitting sounds

Symptoms of nonfluent/agrammatic variant PPA include problems with:

  • omitting words in sentences
  • understanding complex sentences
  • moving the lips and tongue

Sometimes people do not speak at all and may have trouble swallowing as the condition progresses.

Experts do not know the exact cause of PPA, but they believe that proteins within cells start to malfunction in certain areas of the brain, including the frontal and temporal lobes (collectively known as the frontotemporal lobes). Ultimately, this leads to a buildup of abnormal proteins and damage to the brain cells.

The frontotemporal lobes are responsible for language and communication. The same abnormal proteins present in frontotemporal dementia occur in the nonfluent and semantic variants of PPA.

However, the same abnormal proteins present in Alzheimer’s disease are likely responsible for the logopenic variant of PPA.

There is no known cure for PPA, but various treatments can help manage the symptoms and improve a person’s quality of life.

Speech and language therapy is the primary form of treatment for PPA. This therapy aims to:

  • improve communication skills such as speaking, writing, and understanding language
  • teach alternative communication methods such as using gestures and writing notes
  • teach exercises to strengthen the muscles necessary for speech and writing
  • help improve memory and attention

Doctors may also recommend medications to help manage the emotional and behavioral symptoms associated with PPA, such as depression and anxiety.

Learn more about treating aphasia.

The life expectancy of individuals with PPA is highly variable and may depend on several factors, including the subtype and any underlying neurodegenerative disease that may be causing the condition.

The authors of a 2021 study noted that people with the logopenic variant lived for 7.6 years after symptom onset and those with the nonfluent variant lived for 7.1 years after symptom onset. People with the semantic variant survived for 12 years.

However, this study had a small sample size of only 83 people. More research into survival rates for PPA is necessary to confirm these findings and provide more conclusive data.

Getting support is essential for individuals with PPA and their caregivers. Joining a support group that offers emotional support, practical advice, and the opportunity to connect with others may be beneficial.

The National Aphasia Association has a directory of valuable contacts, therapists, and resources for people in the United States who are navigating life with PPA.

Primary progressive aphasia (PPA) is a neurological disorder that affects a person’s communication abilities. It is a language disorder that damages key parts of the brain that are responsible for understanding or producing speech and writing. PPA has three variants, each of which affects different aspects of language.

Currently, there is no cure for PPA, but treatments are available to help manage symptoms. For example, speech and language therapy can help a person communicate, and medications can manage mental health symptoms.

Individuals with PPA and their caregivers should seek support from healthcare professionals, support groups, and other resources. With proper care and management, people with PPA can maintain their connections with others.