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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
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How might hereditary sensory neuropathy type IE be treated?

There is currently no effective treatment for hereditary sensory neuropathy of any type. Management is aimed at injury prevention when sensory impairment is significant; the use of hearing aids and/or assistive communication methods, as needed; and sedative or antipsychotic medications to help reduce the restlessness, roaming behavior, delusions, and hallucinations associated with dementia. Behavioral changes and the loss of insight and judgment can be a significant burden for partners and other caregivers. Information about the progression of the condition and psychological support for these individuals is essential.[1]
Last updated: 10/8/2013

  1. Klein CJ. DNMT1-Related Dementia, Deafness, and Sensory Neuropathy. GeneReviews. May 2012; Accessed 10/8/2013.

Management Guidelines

  • GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.