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Primary Progressive Aphasia

Primary progressive aphasia is a rare neurological syndrome that impairs language capabilities, primarily in people over the age of 50. The exact cause is unknown, but brain imaging studies of people with the condition show shrinkage in the section of the brain devoted to language skills.

Language skills gradually diminish, sometimes leaving the person with great difficulty understanding and using written or spoken language. While there is no cure, training in alternative methods of communication can help compensate for the loss of language skills.

Primary progressive aphasia is sometimes mistaken for Alzheimer's disease because early symptoms of the two disorders can be similar. But primary progressive aphasia is not usually a part of Alzheimer's disease. It is more often linked to frontotemporal dementia — a cluster of related disorders that all originate in the frontal or temporal lobes of the brain.

Doctors aren't sure what causes primary progressive aphasia. Many experts believe that several interacting factors may be involved in the condition, which specifically targets the language center of the brain — located in the brain's left hemisphere.

Brain scans may show a marked shrinkage of the brain's language center in people who have primary progressive aphasia. Blood circulation and brain activity also can be diminished.

Early symptoms of primary progressive aphasia involve difficulty finding the right words during conversations or in writing. Language problems gradually worsen with time. People with this disorder often 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. Many people with this disorder eventually will need help with all their day-to-day care.

There is no specific test that can confirm a diagnosis of primary progressive aphasia. Instead, doctors must rule out other conditions that cause similar symptoms. But first, they must try to determine which language, thinking and memory functions are affected, and to what degree.

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.

Doctors often will turn to family members or close friends to answer such questions as :

  • How is your loved one's emotional state?
  • What is his or her day-to-day routine?
  • Do you know if he or she abuses drugs or alcohol?
  • Is there a history of head trauma?

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.

Magnetic resonance imaging (MRI) or computerized tomography (CT) scans can detect strokes, tumors or other conditions that may have affected the brain.

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.

Language therapy, focusing primarily on practice drills or efforts to compensate for eroding language skills, has shown some success — particularly with comprehension of spoken instructions, production of sentences, retrieval of words and number reading.

The effects of primary progressive aphasia differ greatly among individuals. People who have lost speech skills to a greater extent than reading and writing skills may be able to use a computer to help them communicate. People who have lost speech, reading and writing skills sometimes can learn to use gestures, pantomime and drawing.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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