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.
Symptoms include:
Symptoms include:
Symptoms include:
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.
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:
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.
To diagnose primary progressive aphasia, a neurologist or speech and language pathologist will likely review your symptoms and order tests.
Communication problems that get worse without significant changes in thinking and behavior for a year or two is a hallmark of primary progressive aphasia.
Health care providers might conduct a neurological exam, a speech-language evaluation and a neuropsychological evaluation. Tests will measure your speech, language comprehension and skills. They'll also measure your recognition and naming of objects, recall, and other factors.
Your health care provider might order blood tests to check for infections or look for other medical conditions. Genetic tests can determine if you have genetic changes associated with primary progressive aphasia or other neurological conditions.
A brain MRI can help diagnose primary progressive aphasia. The test can detect shrinking of specific areas of the brain. MRI scans also can detect strokes, tumors or other conditions that affect brain function.
You also might get a positron emission tomography (PET) scan, which shows brain function. This test can uncover problems with glucose metabolism in the areas of the brain that are related to language.
Primary progressive aphasia can't be cured, and there are no medicines to treat it. However, some therapies might help improve or maintain your ability to communicate and manage your condition.
Working with a speech-language pathologist, focusing primarily on ways to make up for lost language skills, can be helpful. Although speech and language therapy can't stop the progression of the condition, it can help you manage your condition. The therapy also may slow the progression of some symptoms.
In cases where symptoms affect movement and balance, working with a physical therapist and occupational therapist can help manage the symptoms.
Losing the ability to communicate can be stressful and frustrating. This is true for the person with primary progressive aphasia and friends and family. If you're a caregiver of someone with primary progressive aphasia, taking these steps can help everyone cope:
Family members eventually may need to make long-term care choices for the person with primary progressive aphasia. They also may need to plan the person's finances and help make legal decisions to prepare for more-serious stages of the condition. Start this process early so the person with primary progressive aphasia can be involved.
Support groups may be offered for caregivers and people with primary progressive aphasia or related conditions. Ask your social worker or other members of your treatment team about community resources or support groups.
If you're experiencing symptoms, you might start by seeing your primary care provider. Your provider may refer you to a doctor trained in brain and nervous system conditions, known as a neurologist, or a speech-language pathologist.
When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:
Take a family member or friend along, if possible. This person can help with communication and to remember the information you receive.
For primary progressive aphasia, some questions to ask your health care provider include:
Don't hesitate to ask other questions.
What happens during your appointment will vary depending on the type of health care provider you see. Your health care provider may ask:
