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Primary progressive aphasia

Definition

Primary progressive aphasia (uh-FAY-zhuh ) is a rare neurological syndrome that impairs language capabilities. People with primary progressive aphasia may have trouble naming objects or may misuse word endings, verb tenses, conjunctions and pronouns.

Symptoms of primary progressive aphasia begin gradually, sometimes before the age of 65, and tend to worsen over time. People with primary progressive aphasia can become mute and may eventually lose the ability to understand written or spoken language.

People with primary progressive aphasia usually continue caring for themselves, working and maintaining their interests, sometimes for many years after the disorder's onset.

Primary progressive aphasia is a type of frontotemporal lobar degeneration, a cluster of related disorders that all originate in the frontal or temporal lobes of the brain.

Symptoms

Primary progressive aphasia symptoms may vary by individual, depending on which portion of the brain's language center is involved. Signs and symptoms may include:

Word-finding pauses in speech
Difficulty in naming objects
Difficulty with comprehension of spoken and written language
Misuse of word endings, verb tenses, conjunctions and pronouns
Inability to comprehend word meanings
Prominent spelling errors

Signs and symptoms may vary depending on the speaking situation. For example, a person may need to pause frequently to find words during a conversation requiring a high level of precision, but then have no pauses when exchanging small talk. Some people with primary progressive aphasia may have less trouble with written language than with spoken language.

Causes

Primary progressive aphasia is caused by a shrinking (atrophy) of the central portion of the brain's left hemisphere — the language center. Scar tissue and abnormal proteins may also be present, and brain activity is often reduced.

Risk factors

Risk factors for primary progressive aphasia include:

Having learning disabilities. People with learning disabilities, particularly dyslexia, are at higher risk of primary progressive aphasia, perhaps because both conditions involve using and understanding language.
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 may be more likely to develop it, too, but a genetic form of primary progressive aphasia is extremely rare.

Complications

People with primary progressive aphasia can become mute and may eventually lose the ability to understand written and spoken language. This generally happens within 10 years of diagnosis. As the disease progresses, other mental skills may become impaired. If this occurs, the affected person eventually will need help with day-to-day care. And depression is common in people who have primary progressive aphasia.

Appointment

You may initially talk to your family doctor about your difficulties in using and understanding language. He or she may refer you to a neurologist or a speech-language pathologist for further evaluation or treatment.

What you can do

Write down all your symptoms, including when each one started and whether any activity or task seems to make them worse.
Make a list of all your medications, including vitamins and supplements.
Take a family member or friend along to help with communication issues.
Write down questions to ask your doctor, including what types of tests you might need and what treatments might be helpful.

What to expect from your doctor What happens during your appointment may vary depending on the type of doctor you're seeing. Your doctor may:

Ask detailed questions about your symptoms
Use written or verbal tests to determine the severity of your communication problems
Test your short-term memory
Order additional medical tests to rule out other causes of aphasia
Talk to someone who knows you well, to get more information about your communication problems and any recent changes in your behavior

Tests and diagnosis

Because there is no specific test to diagnose primary progressive aphasia, your doctor will need to rule out other possible causes of your symptoms.

Communication tests Written and verbal tests pose questions that measure cognitive functions for attention, learning, recall and language. But because these tests depend primarily on language skills, their usefulness declines as the symptoms of primary progressive aphasia worsen.

Blood tests Doctors may order blood tests to check for other factors that can cause memory loss, such as infections, vitamin deficiencies, anemia, medication levels, and disorders of the thyroid, liver or kidneys.

Brain scans MRI or CT scans can detect strokes, tumors or other conditions that may affect brain function. SPECT or PET scans can be helpful if other scans do not show any abnormalities.

Treatments and drugs

Medications There are no drugs that specifically treat primary progressive aphasia. Some doctors have tried Alzheimer's drugs to treat primary progressive aphasia, but no studies have proved these drugs are effective. Experimental therapies will be available with increasing frequency in upcoming years.

Therapy Speech and language therapy, focusing primarily on efforts to compensate for eroding language skills, can be helpful. If speaking and writing skills become limited, examples of alternate communication strategies include: