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Diseases

Genetic and Rare Diseases Information Center (GARD)

Fatal familial insomnia


Other Names for this Disease
  • Familial fatal insomnia
  • Insomnia familial fatal
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Overview

Fatal familial insomnia (FFI) is an inherited prion disease that affects the brain and other parts of the nervous system.[1] Prion diseases, also known as transmissible spongiform encephalopathies (TSE), are a group of rare neurodegenerative conditions that occur when abnormal proteins clump together and accumulate in the brain, leading to tissue damage.[2] The first symptoms of FFI usually begin in mid-life and may include insomnia that worsens over time and vivid dreams when sleep is achieved. These symptoms may be followed by high blood pressure; episodes of hyperventilation; excessive tearing; and/or sexual and urinary tract dysfunction. As the disease progresses, most affected people develop ataxia. FFI usually leads to death within a few months to a few years. Genetic prion diseases are inherited in an autosomal dominant manner and may be caused by mutations in the PRNP gene. Treatment aims at alleviating symptoms when possible.[3]
Last updated: 12/16/2014

References

  1. Prion Disease. Genetics Home Reference. January 2014; http://ghr.nlm.nih.gov/condition/prion-disease.
  2. Prion Diseases. National Institute of Allergy and Infectious Diseases. July 2011; http://www.niaid.nih.gov/topics/prion/Pages/default.aspx.
  3. James A Mastrianni. Genetic Prion Diseases. GeneReviews. January 2, 2014; http://www.ncbi.nlm.nih.gov/books/NBK1229/.
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Basic Information

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • 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 Fatal familial insomnia. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Familial fatal insomnia
  • Insomnia familial fatal
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.