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.
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