Primary Progressive Aphasia

 

 

Primary progressive aphasia (PPA) is a rare 1) ___ syndrome that impairs language capabilities. PPA is a type of frontotemporal lobar degeneration, a cluster of related disorders that all originate in the frontal or temporal lobes of the 2) ___. People with PPA may have trouble naming objects or may misuse word endings, verb tenses, conjunctions and pronouns. Symptoms begin gradually, sometimes before the age of 65, and tend to worsen over time. People can become mute and may eventually lose the ability to understand written or spoken language. People with the disease usually continue caring for themselves, working and maintaining their interests, sometimes for many years after the disorder’s onset.

 

Signs and Symptoms: Sign and symptoms may vary by individual, depending on which portion of the brain’s 3) ___ center is involved and 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 also vary depending on the speaking situation. For example, a person may need to pause frequently to find 4) ___ during a conversation requiring a high level of precision, but then have no pauses when exchanging small talk. Some people may have less trouble with written language than with spoken language.

 

Causes: PPA is caused by a shrinking (atrophy) of the central portion of the brain’s left hemisphere which is 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 5) ___, are at higher risk of PPA, 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 PPA, you may be more likely to develop it, too, but a genetic form of PPA is extremely rare.

 

Complications: People with PPA 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 6) ___ is common in people who have PPA.

 

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 7) ___ 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 8) ___ that specifically treat PPA. Some doctors have tried Alzheimer’s drugs 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:

 

  • 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 or pictures

 

ANSWERS: 1) neurological; 2) brain; 3) language; 4) words; 5) dyslexia; 6) depression; 7) aphasia; 8) drugs

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