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Diseases

Genetic and Rare Diseases Information Center (GARD)

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
  • HSNIE
  • HSAN IE
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My son recently learned that his father and grandmother had mutations in the DNMT1 gene. This gene has been tied to hereditary sensory neuropathy type IE. Do you have information on how this condition might be treated?


Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How might hereditary sensory neuropathy type IE be treated?

There is currently no effective treatment for any type of hereditary sensory neuropathy. Management of symptoms may include:[1][2]
  • meticulous care of the distal limbs, which includes proper fit of shoes, prevention and treatment of callus formation, cleaning and protection of wounds, and avoidance of trauma to the hands and feet
  • injury prevention when sensory impairment is significant
  • the use of hearing aids and/or assistive communication methods as needed
  • sedative or antipsychotic medications to help reduce the restlessness, roaming behavior, delusions, and hallucinations associated with dementia
  • psychological support for caregivers
Last updated: 10/14/2015

References
  • Christopher J Klein. DNMT1-Related Dementia, Deafness, and Sensory Neuropathy. GeneReviews. May 17, 2012; http://www.ncbi.nlm.nih.gov/books/NBK84112/.
  • Robert P Cruse. Hereditary sensory and autonomic neuropathies. UpToDate. Waltham, MA: UpToDate; September, 2015;
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
  • HSNIE
  • HSAN IE
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.