Primary progressive aphasia

Date Updated: 12/27/2018

Overview

Primary progressive aphasia (uh-FAY-zhuh) is a rare nervous system (neurological) syndrome that affects your 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, 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.

This condition progresses slowly, so you may continue caring for yourself and participating in daily life activities for several years after the disorder's onset.

Primary progressive aphasia is a type of frontotemporal dementia, a cluster of related disorders that results from the degeneration of the frontal or temporal lobes of the brain, which include brain tissue involved in speech and language.

Symptoms

Primary progressive aphasia symptoms vary, depending on which portion of the brain's language areas are involved. The condition has three types, which cause different symptoms.

Semantic variant primary progressive aphasia

Signs and symptoms include:

Difficulty comprehending spoken or written language, particularly single words
Trouble comprehending word meanings
Struggling to name objects

Logopenic variant primary progressive aphasia

Signs and symptoms include:

Difficulty retrieving words and word substitutions
Frequently pausing in speech while searching for words
Difficulty repeating phrases or sentences

Nonfluent-agrammatic variant primary progressive aphasia

Signs and symptoms include:

Poor grammar in written and spoken form
Trouble understanding complex sentences
Using grammar incorrectly
May be accompanied by speaking problems such as errors in speech sounds (known as apraxia of speech)

Causes

Primary progressive aphasia is caused by a shrinking (atrophy) of certain sections (lobes) of the brain responsible for speech and language. In this case, the frontal, temporal or parietal lobes, primarily on the left side of the brain, are affected.

Atrophy is associated with the presence of abnormal proteins, and brain activity or function in affected areas might be reduced.

Risk factors

Risk factors for primary progressive aphasia include:

Learning disabilities. If you had a childhood learning disability, particularly developmental dyslexia, you might be at somewhat higher risk of primary progressive aphasia.
Certain gene mutations. Rare gene mutations have been linked to the disorder. If 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. Some people develop substantial difficulty forming sounds to speak (a problem called apraxia of speech), even when their ability to write and comprehend are not significantly impaired.

As the disease progresses, other mental skills, such as memory, can become impaired. Some people develop other neurological symptoms such as problems with movement. With these complications, the affected person eventually will need help with day-to-day care.

People with primary progressive aphasia can also develop depression or behavioral or social problems as the disease progresses. Other problems might include blunted emotions such as unconcern, 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, 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 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

Magnetic resonance imaging (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.

A single-photon emission computerized tomography (SPECT) scan, which creates a 3D image of your brain, or a positron emission tomography (PET) scan, which shows brain function, can uncover blood flow or glucose metabolism abnormalities.

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 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 and may slow the progression of some symptoms.

Coping and support

Losing the ability to communicate can be stressful and frustrating, both 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 consider long-term care options for the person with primary progressive aphasia. They 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 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. 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?