Primary progressive aphasia
DEFINITION
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.
CAUSES
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.
SYMPTOMS
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.
