Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Primary progressive aphasia


Other Names for this Disease

  • Aphasia, primary progressive
  • PPA
  • Primary progressive aphasia syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

Primary progressive aphasia (PPA) is a disorder characterized by language disturbance, including difficulty making or understanding speech (aphasia).[1] In the early stages, PPA often causes difficulty with naming, word finding, or word comprehension. In later stages, affected people often become mute and lose their ability to communicate. PPA may sometimes be the initial manifestation of another neurodegenerative disease, such as frontotemporal dementia or Alzheimer disease.[1] In some cases, PPA is caused by mutations in the GRN gene and is inherited in an autosomal dominant maner.[2]

PPA can be classified into three distinct types which include progressive non-fluent aphasia (PNFA), semantic dementia (SD), and the logopenic variant (LPA).[1]
Last updated: 9/10/2014

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. Hsiung GR & Feldman HH. GRN-Related Frontotemporal Dementia. GeneReview. 2007; http://www.ncbi.nlm.nih.gov/books/NBK1371/. Accessed 4/19/2011.
  3. 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.
Your Questions Answered
by the Genetic and Rare Diseases Information Center

2 question(s) from the public on Primary progressive aphasia have been answered. See questions and answers. You can also submit a new question.

Basic Information

  • The MayoClinic.com provides information about Primary progressive aphasia. Click on the above link to access this information.

In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is a 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.

Insurance Issues

Other Names for this Disease
  • Aphasia, primary progressive
  • PPA
  • Primary progressive aphasia syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.