Primary progressive aphasia

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Definition

Primary progressive aphasia 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, usually 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.

Primary progressive aphasia is a type of frontotemporal dementia, 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 has been damaged. Symptoms may include:

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

In some cases, 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 specifically targets the language center of the brain — located in the brain's left hemisphere. Brain scans typically show a marked shrinkage of the brain's language center in people who have primary progressive aphasia. Brain activity also can be diminished.


Risk factors

Risk factors for primary progressive aphasia include:

* Sex. Men are much more likely to develop the disorder than are women.
* 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.
* Genetics. Several specific gene mutations have been linked to the disorder. If other members of your family have had primary progressive aphasia, you're more likely to develop it, too.


Complications

People with primary progressive aphasia can become mute and may eventually lose the ability to understand written or spoken language. This generally happens within 10 years of diagnosis. As the disease progresses, other mental skills may become impaired. If that occurs, the affected person eventually will need help with their day-to-day care.

Depression is common in people who have primary progressive aphasia.


Preparing for your 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.
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.


Therapy

Speech and language therapy, focusing primarily on efforts to compensate for eroding language skills, has shown some success. If speaking and writing skills become limited, examples of alternate communication strategies include:

* A series of cards that display specific messages, such as common requests
* A word book, used by pointing to the words that can't be articulated
* Laptop computers containing digitally stored words and phrases


Coping and support

Losing the ability to communicate is distressing and incredibly frustrating. Friends and family members can make communication easier by:

* Paying close attention to the affected person
* Giving feedback about the need for clarification
* Providing more time for communication
* Confirming information
* Keeping statements relatively brief
* Supplementing speech with gestures


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Updated: 08/01/2008