Primary progressive aphasia (uh-FAY-zhuh) is a rare nervous system (neurological) syndrome that affects your 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, and worsen over time. People with primary progressive aphasia can lose the ability to speak and write and, eventually, to understand written or spoken language.
This condition progresses slowly, so you may continue caring for yourself and participating in daily life activities for several years after the disorder's onset.
Primary progressive aphasia is a type of frontotemporal dementia, a cluster of related disorders that results from the degeneration of the frontal or temporal lobes of the brain, which include brain tissue involved in speech and language.
Primary progressive aphasia symptoms vary, depending on which portion of the brain's language areas are involved. The condition has three types, which cause different symptoms.
Signs and symptoms include:
Signs and symptoms include:
Signs and symptoms include:
Primary progressive aphasia is caused by a shrinking (atrophy) of certain sections (lobes) of the brain responsible for speech and language. In this case, the frontal, temporal or parietal lobes, primarily on the left side of the brain, are affected.
Atrophy is associated with the presence of abnormal proteins, and brain activity or function in affected areas might be reduced.
Risk factors for primary progressive aphasia include:
People with primary progressive aphasia eventually lose the ability to speak and write, and to understand written and spoken language. Some people develop substantial difficulty forming sounds to speak (a problem called apraxia of speech), even when their ability to write and comprehend are not significantly impaired.
As the disease progresses, other mental skills, such as memory, can become impaired. Some people develop other neurological symptoms such as problems with movement. With these complications, the affected person eventually will need help with day-to-day care.
People with primary progressive aphasia can also develop depression or behavioral or social problems as the disease progresses. Other problems might include blunted emotions such as unconcern, poor judgment or inappropriate social behavior.
To diagnose primary progressive aphasia, your doctor will review your symptoms and order tests.
Worsening communication difficulty without significant changes in thinking and behavior for a year or two is a hallmark of primary progressive aphasia.
Doctors might conduct a neurological examination, a speech-language evaluation and a neuropsychological evaluation. Tests will measure your speech, language comprehension and skills, recognition and naming of objects, recall, and other factors.
Doctors might order blood tests to check for infections or look for other medical conditions. Genetic tests can determine if you have genetic mutations associated with primary progressive aphasia or other neurological conditions.
Magnetic resonance imaging (MRI) scans can help diagnose primary progressive aphasia, detect shrinking of certain areas of the brain and show which area of the brain might be affected. MRI scans can also detect strokes, tumors or other conditions that affect brain function.
A single-photon emission computerized tomography (SPECT) scan, which creates a 3D image of your brain, or a positron emission tomography (PET) scan, which shows brain function, can uncover blood flow or glucose metabolism abnormalities.
Primary progressive aphasia can't be cured, and there are no medications 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 and may slow the progression of some symptoms.
Losing the ability to communicate can be stressful and frustrating, both 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 consider long-term care options for the person with primary progressive aphasia. They may also need to plan the person's finances and help make legal decisions to prepare for more-serious stages of the condition.
Support groups may be available 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. He or she may refer you to a doctor trained in brain and nervous system conditions (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, to help with communication and to help you remember the information you receive.
For primary progressive aphasia, some questions to ask your doctor include:
Don't hesitate to ask other questions.
What happens during your appointment will vary depending on the type of doctor you see. Your doctor may ask:
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