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Primary Progressive Aphasia (PPA)

Fronto-Temporal Degeneration


by André Delacourte

General outline

Primary progressive aphasia (PPA) is a focal dementia characterised by an isolated and gradual dissolution of language function. After several years, this disease develops into fronto-temporal dementia with severe language disorder.

Synonym

Slowly Progressive Aphasia

Symptoms and course

PPA may take a number of forms, it commonly appears initially as a disorder of speaking (an articulatory problem), progressing to nearly total inability to speak in its most severe stage, while comprehension remains relatively preserved. The disease starts with word-finding disturbances (anomia) and frequently proceeds to impair the grammatical structure (syntax) and comprehension (semantics) of language. The speech output in PPA can be fluent or nonfluent. Memory, visual processing, and personality remain relatively well-preserved until the advanced stages and help to distinguish PPA from Alzheimer’s disease.

A less common variety begins with impaired word finding and progressive deterioration of naming and comprehension, with relatively preserved articulation.

Most people with PPA maintain ability to take care of themselves to pursue hobbies and in some instances to remain employed.

Average age of the onset: 50 to 60 years in general. Duration of the disease: several years.

Caregiver problems

People with primary progressive aphasia are fighting against a condition in which they will continue to lose their ability to speak, read, write and/or understand what they hear.

Genetics

There is a family history in about half of all cases of fronto-temporal degeneration. In these families 50 % can be caused by mutation in tau-gene. Some of these inherited forms have been linked to abnormalities on chromosome 3. The causes of non-inherited fronto-temporal dementia are so far unknown.

Frequency

About 10% of fronto-temporal degeneration.

Diagnostic procedures

In order to differentiate FTD from AD, in addition to the clinical assessment, CT and MRI scans may be helpful demonstrating frontal atrophy. Functional imaging (PET, SPECT) in typical cases show frontal / temporal hypometabolism.

Care and treatment

No specific medication. Language rehabilitation has not been tried.


Available services

National Aphasia Association 29 John St., Suite 1103 New York, NY 10038
www.aphasia.org/NAAppa.html


References

  • Sonty, S. P., M. M. Mesulam, et al. (2003). "Primary progressive aphasia: PPA and the language network." Ann Neurol 53(1): 35-49.

 

 
 

Last Updated: Friday 09 October 2009

 

 
  • Acknowledgements

    This information was gathered in the framework of the European Commission financed project "Rare forms of dementia". Neither the European Commission nor any person acting on its behalf is responsible for any use that might be made of the following information.
  • European Union
 
 

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