Primary progressive aphasia

Overview

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.

Symptoms

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 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.
• 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.
• May have problems speaking. This includes making errors in speech sounds, known as apraxia of speech.

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.

When to see a doctor

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.

Causes

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

Risk factors for primary progressive aphasia include:

• Learning disabilities. People who had a childhood learning disability such as dyslexia might be at somewhat higher risk of primary progressive aphasia.
• Certain gene changes. Rare gene changes have been linked to primary progressive aphasia. If other members of your family have had it, you might be more likely to develop it.

Complications

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.

Diagnosis

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.

Neurological exam

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.

Blood tests

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.

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 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.

Treatment

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.

Physical and occupational therapy

In cases where symptoms affect movement and balance, working with a physical therapist and occupational therapist can help manage the symptoms.

Coping and support

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:

• 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 syndrome to others.
• Give the person time to talk.
• Speak slowly in simple, adult sentences and listen carefully.
• 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 treatment team about community resources or support groups.

Preparing for an appointment

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.

What you can do

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:

• Your symptoms, including any that seem unrelated to the reason for your appointment.
• Key personal information, including major stresses, recent life changes and family medical history.
• All medicines, vitamins and supplements you take, including doses.
• Questions to ask your health care provider.

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:

• What's likely causing my symptoms?
• What tests do I need?
• Is my condition likely temporary or chronic?
• 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?
• Should I see a specialist?
• Are there brochures or other printed material I can have? What websites do you recommend?
• Should I consider genetic testing?
• What is going to happen to me over time?

Don't hesitate to ask other questions.

What to expect from your doctor

What happens during your appointment will vary depending on the type of health care provider you see. Your health care provider may ask:

• When did your symptoms begin?
• Have your symptoms been continuous or occasional?
• How bad are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to make your symptoms worse?
• Have your symptoms gotten worse since they started? Have new symptoms emerged?
• Have other family members had similar problems?