Overview
Primary progressive aphasia (PPA) is a rare nervous system syndrome that impacts communication abilities. It affects the ability to express thoughts and understand or find words. Symptoms start gradually, typically before the age of 65, and worsen over time. People with PPA may lose the capacity to speak and write, eventually becoming unable to comprehend written or spoken language. It is a form of frontotemporal dementia, which occurs due to the deterioration of the frontal or temporal lobes of the brain, areas responsible for speech and language. There are three types of PPA: semantic variant, logopenic variant, and nonfluent-agrammatic variant, each with distinct symptoms. If you are experiencing communication difficulties or have concerns about a loved one's communication skills, consult a healthcare provider. In case of sudden changes in speech or communication, seek emergency assistance.
Understanding the disease
Symptoms
Primary progressive aphasia (PPA) is a rare nervous system syndrome that affects the ability to communicate. The symptoms of PPA vary based on which part of the brain's language areas are involved, and the condition has three types.
- Semantic variant primary progressive aphasia: Symptoms include trouble understanding spoken or written language, particularly single words, trouble understanding the meaning of words, not being able to name objects, and trouble formulating sentences.
- Logopenic variant primary progressive aphasia: Symptoms include trouble understanding spoken language, particularly long sentences, pausing and hesitancy during speech while searching for words, and not being able to repeat phrases or sentences.
- Nonfluent-agrammatic variant primary progressive aphasia: Symptoms include poor grammar in written and spoken language, trouble understanding complex sentences, using grammar incorrectly, and potentially having problems speaking, including errors in speech sounds (apraxia of speech). These symptoms develop gradually and get worse over time. People with PPA may continue caring for themselves and participating in daily activities for several years, but eventually lose the ability to speak, write, and understand language.
Risk Factors
The risk factors for developing primary progressive aphasia include learning disabilities and certain gene changes. People who had a childhood learning disability such as dyslexia might be at somewhat higher risk of primary progressive aphasia. Rare gene changes have also been linked to primary progressive aphasia, and if other members of your family have had it, you might be more likely to develop it.
Development
Primary progressive aphasia (PPA) has three types, each with different symptoms, based on the specific areas of the brain's language regions that are affected. The types include:
- Semantic variant PPA: This type is characterized by difficulty in understanding single words, comprehending the meaning of words, and naming objects. People with semantic variant PPA may have trouble forming sentences.
- Logopenic variant PPA: This type is marked by difficulty in understanding long sentences and complex language. People with logopenic variant PPA often exhibit pauses and hesitations during speech while searching for words and may struggle to repeat phrases or sentences.
- Nonfluent-agrammatic variant PPA: This type is characterized by poor grammar in both written and spoken language. People with nonfluent-agrammatic variant PPA have trouble understanding complex sentences and often use incorrect grammar. These types of primary progressive aphasia develop as a result of the atrophy of specific lobes in the brain, primarily the left side, which is responsible for speech and language. The atrophy is associated with the presence of certain proteins in the brain that may reduce brain activity or function. Genetic factors, such as rare gene changes, can also play a role in the development of PPA.
Assessment and Diagnosis
Triage
You should see a healthcare provider if you have concerns about your ability to communicate or if you notice symptoms of primary progressive aphasia in a family member or friend. If the changes in speech or communication occur suddenly, it is important to call 911 or your local emergency number.
Diagnosis
To diagnose primary progressive aphasia, a healthcare provider will likely review your symptoms and order tests, including a neurological exam, speech-language evaluation, and neuropsychological evaluation. These tests will measure your speech, language comprehension, and skills, as well as your recognition and naming of objects, recall, and other factors. Blood tests may be ordered to check for infections or other medical conditions, and genetic tests can determine if there are genetic changes associated with primary progressive aphasia or other neurological conditions. A brain MRI can help diagnose primary progressive aphasia by detecting shrinking of specific areas of the brain, and a PET scan can show brain function and uncover problems with glucose metabolism in the language-related areas of the brain. There is no cure for primary progressive aphasia, but speech and language therapy can help improve or maintain your ability to communicate and manage the condition.
Management and Treatment
Complications
Primary progressive aphasia can lead to several complications as the disease progresses. These may include:
- Difficulty with speaking and writing: People with primary progressive aphasia eventually lose the ability to speak and write, which may take anywhere from 3 to 15 years. They also have trouble understanding written and spoken language. Some people may not be able to form sounds to speak, even when they still have the ability to write and comprehend language, which is known as apraxia of speech.
- Impact on mental skills: As the disease advances, other mental skills such as memory, planning, and organizing can be affected. Some people may develop additional symptoms, including problems with movement, balance, and swallowing. These complications may require assistance with daily care.
- Nonfluent-agrammatic variant primary progressive aphasia: This specific variant of the disorder is characterized by poor grammar in both written and spoken language, trouble understanding complex sentences, and using incorrect grammar. People with this type of primary progressive aphasia may have problems speaking, including making errors in speech sounds or having trouble saying words quickly.
- Depression and emotional changes: As the disease progresses, individuals with primary progressive aphasia may develop depression or exhibit blunted emotions, such as showing no concern. Poor judgment or inappropriate social behavior may also occur. It is essential to consult a healthcare provider if you have concerns about your ability to communicate or if you notice symptoms in a family member or friend.
Treatment
Unfortunately, at this time, there is no cure for primary progressive aphasia. Treatment is primarily focused on managing symptoms and providing support for individuals with the condition. Speech and language therapy can help improve communication skills and slow the progression of speech and language difficulties. Other forms of therapy, such as physical therapy, occupational therapy, and cognitive-behavioral therapy, may also be beneficial in managing specific symptoms and maintaining daily living skills. Medications may be prescribed to address specific symptoms or complications, such as depression or anxiety. It is important to consult a healthcare provider for personalized advice and management strategies for primary progressive aphasia.
Preparing for medical consultation
To prepare for an appointment for primary progressive aphasia, you should:
- Make a list of your symptoms, including any that seem unrelated to the reason for your appointment.
- Gather key personal information, such as major stresses, recent life changes, and family medical history.
- Bring a list of all medicines, vitamins, and supplements you take, including doses.
- Prepare a list of questions to ask your healthcare provider.
- Consider bringing a family member or friend to help with communication and remembering information. Before attending the appointment, it's essential to be patient and take the time to communicate clearly. Remember that primary progressive aphasia can affect communication, so it's essential to be understanding and take care of your personal needs. Support groups may also be available for caregivers and people with primary progressive aphasia or related conditions.