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Genetic and Rare Diseases Information Center (GARD)

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Hereditary sensory neuropathy type IE


Other Names for this Disease

  • DNMT1-Related Dementia, Deafness, and Sensory Neuropathy
  • Hereditary sensory and autonomic neuropathy type IE
  • Hereditary sensory neuropathy with hearing loss and dementia
  • HSAN IE
  • HSNIE
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Overview

Hereditary sensory neuropathy type IE (HSNIE) is a degenerative disorder of the central and peripheral the nervous systems. It is characterized by sensory impairment of the feet and lower legs (affecting sensations such as pain, temperature, and touch), loss of the ability to sweat (sudomotor function) in the hands and feet, sensorineural hearing loss, and gradual loss of intellectual function (dementia). Symptoms typically begin in the twenties or thirties, although age of onset and symptom severity are variable, even within families. HSNIE is caused by mutations in the DNMT1 gene. This condition is inherited in an autosomal dominant pattern.[1][2]
Last updated: 10/8/2013

References

  1. Hereditary sensory and autonomic neuropathy type IE. Genetics Home Reference (GHR). November 2012; http://ghr.nlm.nih.gov/condition/hereditary-sensory-and-autonomic-neuropathy-type-ie. Accessed 10/8/2013.
  2. Klein CJ. DNMT1-Related Dementia, Deafness, and Sensory Neuropathy. GeneReviews. May 2012; http://www.ncbi.nlm.nih.gov/books/NBK84112/. Accessed 10/8/2013.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Hereditary sensory neuropathy type IE. This website is maintained by the National Library of Medicine.

In Depth Information

  • 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 Hereditary sensory neuropathy type IE. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • DNMT1-Related Dementia, Deafness, and Sensory Neuropathy
  • Hereditary sensory and autonomic neuropathy type IE
  • Hereditary sensory neuropathy with hearing loss and dementia
  • HSAN IE
  • HSNIE
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.