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
Last Updated: Friday 09 October 2009
