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Primary progressive aphasia

Overview

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.

Functions of the brain

Different areas of the brain are responsible for different vital functions.

Symptoms

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.

Semantic variant primary progressive aphasia

Symptoms include these difficulties:

  • Comprehending spoken or written language, particularly single words
  • Comprehending word meanings
  • Naming objects

Logopenic variant primary progressive aphasia

Symptoms include:

  • Having difficulty retrieving words
  • Frequently pausing in speech while searching for words
  • Having difficulty repeating phrases or sentences

Nonfluent-agrammatic variant primary progressive aphasia

Symptoms include:

  • Having difficulty forming words
  • Being hesitant and halting in speech
  • Making errors in speech sounds
  • Having difficulty understanding sentences
  • Using grammar incorrectly

Causes

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.

Risk factors

Risk factors for primary progressive aphasia include:

  • Having learning disabilities. A learning disability might put you at somewhat higher risk of primary progressive aphasia.
  • Having certain gene mutations. Rare gene mutations have been linked to the disorder. If several other members of your family have had primary progressive aphasia, you might be more likely to develop it.

Complications

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.

Diagnosis

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.

Neurological examination

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.

Blood tests

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.

Brain scans

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.

Treatment

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.

Speech and language therapy

Working with a speech-language pathologist, focusing primarily on efforts to compensate for eroding language skills, can be helpful. Although speech and language therapy hasn't been proved to slow progression of the condition, it can help you manage your condition.

Preparing for an appointment

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.

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 medications, vitamins and supplements you take, including doses
  • Questions to ask your doctor

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:

  • What's likely causing my symptoms?
  • Other than the most likely cause, what are other possible causes?
  • What tests do I need?
  • Is my condition likely temporary or chronic?
  • What's the best course of action?
  • What are the alternatives 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? 

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 doctor you see. Your doctor may ask:

  • When did your symptoms begin?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

Coping and support

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:

  • 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
  • Take care of your personal needs — get enough rest and make time for social activities

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.

Last updated: December 22nd, 2016

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