Print friendly version
Primary progressive aphasia
Other Names for this Disease
- Aphasia, primary progressive
- PPA
- Primary progressive aphasia syndrome
Related Diseases
More Related DiseasesSee 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 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]
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]
References
- Ratnavalli E.. Progress in the last decade in our understanding of primary progressive aphasia. Ann Indian Acad Neurol. 2010. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3039160/?tool=pubmed. Accessed February 20, 2013.
- NINDS Frontotemporal Dementia Information Page. National Institute of Neurological Disorders and Stroke (NINDS). http://www.ninds.nih.gov/disorders/picks/picks.htm. Accessed April 19, 2011.
- Hsiung GR & Feldman HH. GRN-Related Frontotemporal Dementia. GeneReview. http://www.ncbi.nlm.nih.gov/books/NBK1371/. Accessed April 19, 2011.
Your Questions Answered
by the Genetic and Rare Diseases Information Center1 question(s) from the public on Primary progressive aphasia have been answered. See questions and answers. You can also submit a new question.
On this page
General Information
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic.
- 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.
- The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Primary progressive aphasia. Click on the link to go to OMIM and review these resources.
Insurance Issues
- The Social Security Administration has included this condition in their Compassionate Allowances Initiative. This initiative speeds up the processing of disability claims for applicants with certain medical conditions that cause severe disability. More information about Compassionate Allowances and applying for Social Security disability is available online.