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Genetic and Rare Diseases Information Center (GARD)

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Primary progressive aphasia


Other Names for this Disease
  • Aphasia, primary progressive
  • PPA
  • Primary progressive aphasia syndrome
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Overview


Primary progressive aphasia (PPA) is a type of dementia characterized by language disturbance, including difficulty making or understanding speech (aphasia). PPA can be part of frontotemporal dementia or Alzheimer disease.[1] Spatial skills and memory usually remain intact.[2] In early stages, PPA often manifests as deficits in naming, word finding, or word comprehension. In late stages, affected individuals often become mute and lose their ability to communicate. In some cases, PPA is inherited in an autosomal dominant fashion and caused by mutations in the GRN gene.[3]

PPA can be classified into three distinct variants based on language profiles, which include progressive non-fluent aphasia (PNFA), semantic dementia (SD), and the recently characterized logopenic variant (LPA).[1]
Last updated: 8/22/2011

References

  1. Ratnavalli E.. Progress in the last decade in our understanding of primary progressive aphasia. Ann Indian Acad Neurol. 2010; 13:S109-S115. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3039160/?tool=pubmed. Accessed 2/20/2013.
  2. NINDS Frontotemporal Dementia Information Page. National Institute of Neurological Disorders and Stroke (NINDS). 2011; http://www.ninds.nih.gov/disorders/picks/picks.htm. Accessed 4/19/2011.
  3. Hsiung GR & Feldman HH. GRN-Related Frontotemporal Dementia. GeneReview. 2007; http://www.ncbi.nlm.nih.gov/books/NBK1371/. Accessed 4/19/2011.
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In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Primary progressive aphasia. Click on the link to view a sample search on this topic.

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