Hereditary sensory neuropathy type IE
Other Names for this Disease
- Hereditary sensory neuropathy with hearing loss and dementia
- Hereditary sensory and autonomic neuropathy type IE
- DNMT1-Related Dementia, Deafness, and Sensory Neuropathy
- HSAN IE
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nervous systems. Symptoms typically begin by age 20 to 35 and include sensory impairment of the lower legs and feet; loss of sweating in the hands and feet; sensorineural hearing loss; and gradual decline of mental ability (dementia). The severity of symptoms and age of onset vary, even within the same family. HSNIE is caused by a mutation in the DNMT1 gene and is inherited in an autosomal dominant manner. There is no effective treatment, but management may include injury prevention, the use of hearing aids, and sedative or antipsychotic medications for symptoms of dementia.Hereditary sensory neuropathy type IE (HSNIE) is a progressive disorder of the central and peripheral
Last updated: 10/14/2015
- 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.
- Christopher J Klein. DNMT1-Related Dementia, Deafness, and Sensory Neuropathy. GeneReviews. May 17, 2012; http://www.ncbi.nlm.nih.gov/books/NBK84112/.
- Genetics Home Reference (GHR) contains information on Hereditary sensory neuropathy type IE. This website is maintained by the National Library of Medicine.
- The National Institute of Neurological Disorders and Stroke (NINDS) (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- 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.