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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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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 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