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Primary progressive aphasia

Primary progressive aphasia (uh-FAY-zhuh) is a rare nervous system (neurological) syndrome that impairs language capabilities. People with primary progressive aphasia may have trouble expressing their thoughts and comprehending or finding words.

Symptoms of primary progressive aphasia begin gradually, sometimes before age 65, and tend to worsen over time. People with primary progressive aphasia can become mute and may eventually lose the ability to understand written or spoken language.

People with primary progressive aphasia may continue caring for themselves and participating in daily life activities for several years after the disorder's onset, as the condition progresses slowly.

Primary progressive aphasia is a type of frontotemporal degeneration, a cluster of related disorders that originate in the frontal or temporal lobes of the brain.

Symptoms Causes Risk factors Complications

Primary progressive aphasia symptoms may vary by individual, depending on which portion of the brain's language center is involved.

Primary progressive aphasia has three types, which cause different symptoms.

Semantic variant primary progressive aphasia


In this condition, you may experience symptoms such as:

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

Lopogenic variant primary progressive aphasia


In this condition, you may experience symptoms such as:

  • Difficulty retrieving correct words in speech
  • Frequent pauses in your speech while searching for words
  • Slow speech
  • Difficulty repeating phrases or sentences

Nonfluent-agrammatic variant primary progressive aphasia


In this condition, you may experience symptoms such as:

  • Difficulty speaking
  • Hesitant, halting speech
  • Making errors in speech sounds
  • Difficulty understanding sentences
  • Using grammar incorrectly

Symptoms may vary depending on the speaking situation and the type of primary progressive aphasia. For example, a person may need to pause frequently to find words during a conversation requiring a high level of precision but then have no pauses when exchanging small talk. Reading and writing also are usually affected.

Primary progressive aphasia is caused by a shrinking (atrophy) of the frontal and temporal lobes in the brain, primarily on the left side of the brain. Primary progressive aphasia affects the language center in your brain. Scar tissue and abnormal proteins also may be present, and brain activity is often reduced.

Risk factors for primary progressive aphasia include:

  • Having learning disabilities. People with learning disabilities, particularly dyslexia, may be at higher risk of primary progressive aphasia, perhaps because both conditions involve using and understanding language.
  • Having certain gene mutations. Rare gene mutations have been linked to the disorder. If several other members of your family have had primary progressive aphasia, you may be more likely to develop it.

People with primary progressive aphasia may become mute and may eventually lose the ability to understand written and spoken language.

As the disease progresses, other mental skills, such as memory, may become impaired. Some people may develop other neurological conditions over time. If these complications occur, the affected person eventually will need help with day-to-day care.

People with primary progressive aphasia may also develop behavioral or social problems, such as being anxious or irritable. Other problems may include blunted emotions, poor judgment or inappropriate social behavior.

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