Primary progressive aphasia (uh-FAY-zhuh) is a rare nervous system (neurological) syndrome that affects your ability to communicate. People with primary progressive aphasia 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.
People with this condition may continue caring for themselves and participating in daily life activities for several years after the disorder's onset because it 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.
Primary progressive aphasia symptoms vary, depending on which portion of the brain's language area is involved. The condition has three types, which cause different symptoms.
Symptoms include these difficulties:
Primary progressive aphasia is caused by a shrinking (atrophy) of the frontal, temporal or parietal lobes in the brain, primarily on the left side. The condition affects the language centers in your brain.
Scar tissue and abnormal proteins also might be present, and brain activity might be reduced.
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 as well as 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, measure medication levels or look for other medical conditions. Genetic tests can determine if you have genetic mutations associated with primary progressive aphasia or other neurological conditions.
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.
Single-photon emission computerized tomography or PET scans can show blood flow or glucose metabolism abnormalities in areas of your brain.
People with primary progressive aphasia eventually lose the ability to speak and write, and to understand written and spoken language.
As the disease progresses, other mental skills, such as memory, can become impaired. Some people develop other neurological conditions. With these complications, the affected person eventually will need help with day-to-day care.
People with primary progressive aphasia can also develop behavioral or social problems as the disease progresses, such as anxiety or irritability. Other problems might include blunted emotions, poor judgment or inappropriate social behavior.
Losing the ability to communicate is distressing and incredibly frustrating, both for the person with primary progressive aphasia and for friends and family. To help everyone involved cope:
Family members eventually may need to consider long-term care options for the person with primary progressive aphasia. Family members 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 you and the person with primary progressive aphasia or related conditions. Ask your social worker or other members of your treatment team about community resources or support groups.
Risk factors for primary progressive aphasia include: