Primary Progressive Aphasia

What Is Primary Progressive Aphasia?

Primary progressive aphasia is a form of degenerative neurological disorder, in which the first signs are changes in ability to communicate. In other forms of dementia, language may be relatively spared until late in the process. In primary progressive aphasia, language is affected first, and there may be other effects later in the disease process.

Some people with primary progressive aphasia have a family history of this disorder. Others do not. The cause of primary progressive aphasia is unknown. Since primary progressive aphasia is diagnosed on the basis of symptoms, it is possible that there is more than one cause of the disorder.

Symptoms Of Progressive Aphasia

Slowly progressive loss of ability to speak and /or understand others
The ability to find words in conversation, name items, or understand complex speech may be affected.
No other cause can be found for the changes in language ability.

Types Of Primary Progressive Aphasia

There are three subtypes of primary progressive aphasia.

1. Semantic primary progressive aphasia

  • The person speaks fluently, but may use simpler, less precise words than they normally would.
  • They may have difficulty remembering names.
  • The person may have difficulty understanding less common words.
  • Has increased difficulty reading and writing, especially words with irregular spelling
  • Has increased difficulty recognizing people or objects
  • Brain scans show deterioration of the ventral inferior temporal lobe

2. Nonfluent or agrammatic primary progressive aphasia

  • Slow, effortful speech
  • May leave out the small, grammatical words in the sentence, or grammatical endings
  • Comprehension is good, or only has difficulty with complex sentences.
  • Brain scans show deterioration of the left posterior fronto-insular area, and possibly
    the supplemental motor area

3. Logopenic primary progressive aphasia

  • Speaks fluently, but has difficulty recalling words AND has difficulty repeating information.
  • May make sound errors or substitute sounds when saying words
  • May leave out grammatical words or endings
  • Comprehension is usually good
  • Brain scans show deterioration of the left posterior perisylvan or parietal lobe

Onset And Prognosis

  • Primary progressive aphasia can begin at any time, from age 17 to age 81. However, the typical age of onset is about 60 years old.
  • Men are diagnosed about twice as often as women.
  • After diagnosis, people can live anywhere from one year to 20 years. 75% are still alive five years after diagnosis. Less than 20% are still alive 10 years after diagnosis.
  • About half of the people described in scientific articles developed cognitive deficits with time.

Treatment Of Primary Progressive Aphasia

Presently, there is no cure for primary progressive aphasia. Treatment is to maintain function for as long as possible, and to make life easier for the person affected and those close to them.

Talking to others, being socially active, and playing word games wil maintain function for as long as possible.

It is recommended that the person affected and those close to them become familiar with augmentive/alternative communication (AAC) early. It will then be easier to use when it is needed as the disease process progresses.

AAC can be simple, such as a notebook or heavy sheet of paper with frequently needed phrases or words. It is helpful to pair the written words with pictures to make them easier to recognize.

There are electronic forms of AAC. Smart phone or tablet apps that can used as AAC.

There are also dedicated devices. These are larger and more expensive. Insurance may cover it as a medically necessary device.

Support Groups For Primary Progressive Aphasia

Facebook support group for primary progressive aphasia

This private Facebook group has 3,190 members as of this writing. Members include people diagnosed with primary progressive aphasia, involved family members and caregivers, and professionals.
You must have a Facebook account to sign up for the group.

https://www.facebook.com/groups/441592592551251/

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Minnesota Connect Aphasia Now “Staying Connected” Primary Progressive Aphasia Program

Minneapolis, Minnesota
8 week program for people with primary progressive aphasia.
There is an ongoing support program for those who complete the 8 week program.
https://mncan.org/primary-progressive-aphasia-ppa-program/

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Northwestern University, Chicago

The Feinberg School of Medicine at Northwestern University in Evanston, Illinois has the Mesulam Center for Cognitive Neurology and Alzheimer’s Disease. They provide treatment, research and support groups for caregivers of people with primary progressive aphasia and other dementias.

For more information, go to

https://www.brain.northwestern.edu/dementia/ppa/index.html

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Rare Dementias Support Group, University College of London, United Kingdom

The Rare Dementias Support Group offers support groups for primary progressive aphasia and other rare dementias in various locations around England.

They have informational videos at this site

https://www.raredementiasupport.org/primary-progressive-aphasia/

There is an older site that has not been updated since 2016, but you can watch Youtube videos of previously held meetings.

https://www.ucl.ac.uk/drc/support-groups/ppa

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Australian Fronto-Temporal Dementia Association, Australia

They have two types of groups. Some groups are run by professionals and offer support and advice. Other groups are run by experienced caregivers, and offer a mix of support and social activities. Groups meet in various locations around Australia.

For more information, go to https://theaftd.org.au/support-groups/

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International PPA Connection

A German website in the German language
https://www.ppaconnection.org/