Clinical phenotypes of progressive aphasia. (March 2009)

• Record Type: Journal Article
• Authors: Cappa, Stefano F; Gorno-Tempini, Maria Luisa
• Is Part Of: Future neurology. Volume 4:Number 2(2009)
• Page Start: 153
• Page End: 160
• Publication Date: 2009-03
• Subjects: dementia -- language -- Pick''s disease -- progranulin -- tauopathies; Neurology -- Research -- Periodicals; 611.8005
• DOI: 10.2217/14796708.4.2.153
• Languages: English
• ISSNs: 1479-6708

Abstract

Primary progressive aphasia is defined as an insidious, gradual impairment of language function, which is not accompanied by other cognitive disorders for at least 2 years after onset, and which can be due to a number of neurodegenerative disorders, such as the different varieties of frontotemporal dementia or Alzheimer''s disease. The clinical presentations are heterogeneous, as they faithfully reflect the preferential location of pathological involvement within the language networks. A careful definition of the clinical phenotypes of primary progressive aphasia is contributing to the understanding of language organization in the brain. Moreover, the predictive value of the clinical phenotype as to the underlying pathology has important implications for clinical trials of disease-modifying drugs.