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
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 QuestionMy 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?
We have identified the following information that we hope you find helpful. If you still have questions, please contact us.
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.
Last updated: 10/8/2013
- Klein CJ. DNMT1-Related Dementia, Deafness, and Sensory Neuropathy. GeneReviews. May 2012; http://www.ncbi.nlm.nih.gov/books/NBK84112/. Accessed 10/8/2013.