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
- HSAN IE
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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;
- 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.