Primary progressive aphasia

Primary progressive aphasia (uh-FAY-zhuh) is a rare nervous system condition that affects a person's ability to communicate. People who have primary progressive aphasia can have trouble expressing their thoughts and understanding or finding words.

Symptoms develop 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 conditions that result from the degeneration of the frontal or temporal lobes of the brain. These areas include brain tissue involved in speech and language. Not all people with primary progressive aphasia have dementia, but most develop it. The term "dementia" is typically not used until a person can't do things alone due to changes in their thinking and understanding.

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

Symptoms include:

• Trouble understanding spoken or written language, particularly single words.
• Trouble understanding the meaning of words.
• Not being able to name objects.
• Trouble spelling words, especially those that aren't spelled the way that they sound, such as the word "yacht."

Logopenic variant primary progressive aphasia

Symptoms include:

• Trouble understanding spoken language, particularly long sentences.
• Pausing and hesitating to search for words while speaking.
• Not being able to repeat phrases or sentences.

Nonfluent-agrammatic variant primary progressive aphasia

Symptoms include:

• Leaving out words or putting words in the wrong order in written and spoken language.
• Trouble understanding complex sentences.
• Trouble speaking. This includes making errors in speech sounds, known as apraxia of speech.

Primary progressive apraxia of speech is related to primary progressive aphasia. However, people with this condition don't have trouble with language. They have problems speaking. This includes making errors in speech sounds or having trouble saying words quickly.

When to see a doctor

See your healthcare professional 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 healthcare professional.

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 primary progressive aphasia, 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 happens on the left side of the brain. The areas affected are responsible for speech and language.

Atrophy is linked to 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 may have a higher risk of developing primary progressive aphasia.
• Certain gene changes. Although primary progressive aphasia most often happens randomly, rare gene changes have been linked to the condition. If other members of your family have had primary progressive aphasia, consider genetic testing to see if you are 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. People with the condition also have trouble understanding written and spoken language.

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 need help with day-to-day care.

People with primary progressive aphasia also can develop depression as the disease progresses. Other complications might include blunted emotions such as not showing concern, poor judgment or social behavior that's not appropriate.

There is no known way to prevent primary progressive aphasia. However, you can keep your brain healthy by using GROWTH:

• Get quality sleep.
• Reduce stress.
• Open connections.
• Work out.
• Try new things.
• Healthy eating.

To diagnose primary progressive aphasia, a neurologist or speech and language pathologist will likely review your symptoms and order tests.

Communication troubles that get worse without significant changes in thinking and behavior for 2 to 3 years are a hallmark of primary progressive aphasia.

Tests and procedures used to diagnose primary progressive aphasia may include:

Neurological exam

Healthcare professionals might conduct a neurological exam, a speech-language evaluation and a neuropsychological evaluation. Tests measure speech, language comprehension and skills. They also measure your recognition and naming of objects, recall, and other factors.

Blood and body fluid tests

Your healthcare professional might order blood tests or a lumbar puncture, also known as a spinal tap, to check for infections or look for other medical conditions. Genetic tests can determine whether you have gene changes linked to primary progressive aphasia or other neurological conditions.

Brain scans

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 and other conditions that affect brain function.

You also might get a fluorodeoxyglucose positron emission tomography scan, also known as a FDG PET scan, which shows brain function. This test can show changes in how the brain uses glucose in the areas that are related to language. You also may have other PET scans to look for the presence of certain proteins linked to Alzheimer's disease.

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.

Speech and language therapy

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. Your therapist may recommend that your caregivers also attend the appointments.

Physical and occupational therapy

If your symptoms affect movement and balance, working with a physical therapist and an occupational therapist can help manage the symptoms.

Losing the ability to communicate can be stressful and frustrating. This is true for both the person with primary progressive aphasia and for friends and family. If you're a caregiver of someone with primary progressive aphasia, taking these steps can help everyone cope:

• Learn all you can about the condition.
• Have the person with the condition carry an identification card and other materials that can help explain the condition to others.
• Give the person time to talk.
• Speak slowly in simple, adult sentences and listen carefully.
• Talk with the person with the condition about how you can help.
• Take care of your personal needs. Get enough rest and make time for social activities.

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 healthcare team about community resources or support groups. Other sources of information include the National Aphasia Association and the Association for Frontotemporal Degeneration.

If you're experiencing symptoms, you might start by seeing your primary healthcare professional. This person may refer you to a doctor trained in brain and nervous system conditions, known as a neurologist. You also may be referred to a speech-language pathologist.

What you can do

Here's some information to help you get ready for your appointment.

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins and supplements you're taking and the doses.
• Take a family member or friend along. Sometimes it can be very hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

For primary progressive aphasia, some questions to ask might include:

• What's likely causing my symptoms?
• What tests do I need?
• Is my condition likely temporary or ongoing?
• Should I see a specialist?
• Should I consider genetic testing?
• Are there brochures or other printed material I can have? What websites do you recommend?
• What's the best course of action?
• Are there other choices to the primary approach you're suggesting?
• I have other health conditions. How can I best manage them together?
• Are there restrictions I need to follow?
• What is going to happen to me over time?

Don't hesitate to ask other questions.

What to expect from your doctor

Your healthcare professional is likely to ask you several questions, such as:

• When did your symptoms begin?
• What symptoms came first?
• Have you had any new symptoms?
• Have your symptoms come and gone or stayed steady?
• How bad are your symptoms?
• Have your symptoms gotten worse?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to make your symptoms worse?
• Have other family members had similar conditions?