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
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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?
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There is currently no effective treatment for any type of hereditary sensory neuropathy. Management of symptoms may include:
- 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
- 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;