Primary progressive aphasia (uh-FAY-zhuh) is a rare nervous system syndrome that affects the ability to communicate. People who have it can have trouble expressing their thoughts and understanding or finding words.
Symptoms begin gradually, often before age 65. They get worse over time. People with primary progressive aphasia can lose the ability to speak and write. Eventually they're not able to understand written or spoken language.
This condition progresses slowly. People who have primary progressive aphasia may continue caring for themselves and participating in daily activities for several years.
Primary progressive aphasia is a type of frontotemporal dementia. Frontotemporal dementia is a cluster of disorders that results from the degeneration of the frontal or temporal lobes of the brain. These areas include brain tissue involved in speech and language.
Primary progressive aphasia symptoms vary based on which part of the brain's language areas are involved. The condition has three types. Each type causes different symptoms. Symptoms develop over time and gradually get worse.
Semantic variant primary progressive aphasia
- Trouble understanding spoken or written language, particularly single words.
- Trouble understanding the meaning of words.
- Not being able to name objects.
- Trouble formulating sentences.
Logopenic variant primary progressive aphasia
- Trouble understanding spoken language, particularly long sentences.
- Pausing and hesitancy during speech while searching for words.
- Not being able to repeat phrases or sentences.
Nonfluent-agrammatic variant primary progressive aphasia
- Poor grammar in written and spoken language.
- Trouble understanding complex sentences.
- Using grammar incorrectly.
- May have problems speaking. This includes making errors in speech sounds, known as apraxia of speech.
Primary progressive apraxia of speech is related to primary progressive aphasia, but people with this disorder don't have trouble with language. They have problems speaking. This includes making errors in speech sound or having trouble saying words quickly.
When to see a doctor
See your health care provider if you have concerns about your ability to communicate. If you have a family member or friend who has symptoms of primary progressive aphasia, talk to the person about your concerns. Offer to go with the person to see a health care provider.
If changes in speech or communication come on suddenly, call 911 or your local emergency number.
Primary progressive aphasia is caused by a shrinking of certain areas of the brain, known as lobes. In this case, the frontal, temporal or parietal lobes are affected. When areas of the brain shrink, it's called atrophy. The atrophy caused by primary progressive aphasia mainly occurs on the left side of the brain. The areas affected are responsible for speech and language.
Atrophy is associated with the presence of certain proteins in the brain. The proteins may reduce brain activity or function.
Risk factors for primary progressive aphasia include:
- Learning disabilities. People who had a childhood learning disability such as dyslexia might be at somewhat higher risk of primary progressive aphasia.
- Certain gene changes. Rare gene changes have been linked to primary progressive aphasia. If other members of your family have had it, you might be more likely to develop it.
People with primary progressive aphasia eventually lose the ability to speak and write. This may take anywhere from 3 to 15 years. They also have trouble understanding written and spoken language. Some people are not able to form sounds to speak, even when they still have the ability to write and comprehend language. This is called apraxia of speech.
As the disease progresses, other mental skills such as memory, planning and organizing can be affected. Some people develop other symptoms such as problems with movement, balance and swallowing. With these complications, people with the disease eventually will need help with day-to-day care.
People with primary progressive aphasia also can develop depression as the disease progresses. Other problems might include blunted emotions such as not showing concern, poor judgment or inappropriate social behavior.