Hereditary Sensory Neuropathy Type I
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Hereditary Sensory Neuropathy Type I is not the name you expected.
Hereditary sensory neuropathy type I (HSN1) belongs to a group of similar but distinct genetic disorders characterized by abnormalities affecting the nerves, especially of those of the hands and feet. These degenerative disorders of the nervous system (neurodegenerative disorders) are slowly progressive and predominantly affect the sensory nerves, which frequently leads to loss of feeling (sensation) in the hands and feet. This sensory loss is due to abnormal functioning of the sensory nerves that control responses to pain and temperature and may also affect the autonomic nervous system that controls other involuntary or automatic body processes. Specific symptoms can vary widely from one person to another. HSN1 occurs due to mutations in specific genes and is inherited as an autosomal dominant trait. There are several subtypes of HSN1 designated A through E, each one associated with a different gene.
The hereditary sensory neuropathies (HSNs), also known as the hereditary sensory and autonomic neuropathies, include at least six similar, but distinct inherited degenerative disorders of the nervous system (neurodegenerative) that frequently progress to loss of feeling, especially in the hands and feet. Some of these disorders have several subtypes based upon the specific associated genes. The classification of the HSNs is complicated, and the experts do not always agree on it. Furthermore, HSNs are classified more broadly as peripheral neuropathies or disorders of the peripheral nervous system, which encompasses all of the nerves outside of the central nervous system. NORD's Rare Disease Database has separate reports on HSN2, HSN3 (which is related to, or identical with familial dysautonomia), and HSN4.
Center for Peripheral Neuropathy
- University of Chicago
- 5841 South Maryland Ave, MC 2030
- Chicago, IL 60637
- Tel: (773)702-5659
- Fax: (773)702-5577
- Website: http://peripheralneuropathycenter.uchicago.edu/
Foundation for Peripheral Neuropathy
- 485 Half Day Road
- Suite 350
- Buffalo Grove, IL 60089
- Tel: (847)883-9942
- Fax: (847)883-9960
- Tel: (877)833-9942
- Email: email@example.com
- Website: http://www.foundationforpn.org
Genetic and Rare Diseases (GARD) Information Center
- PO Box 8126
- Gaithersburg, MD 20898-8126
- Tel: (301)251-4925
- Fax: (301)251-4911
- Tel: (888)205-2311
- Website: http://rarediseases.info.nih.gov/GARD/
NIH/National Institute of Neurological Disorders and Stroke
- P.O. Box 5801
- Bethesda, MD 20824
- Tel: (301)496-5751
- Fax: (301)402-2186
- Tel: (800)352-9424
- Website: http://www.ninds.nih.gov/
- 60 East 42nd Street
- Suite 942
- New York, NY 10165
- Tel: (212)692-0662
- Fax: (212)692-0668
- Email: firstname.lastname@example.org
- Website: http://www.neuropathy.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.
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This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
Last Updated: 7/11/2014
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