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. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
- Hereditary Sensory and Autonomic Neuropathy (HSAN) Type I
- Hereditary Sensory Radicular Neuropathy, Autosomal Dominant
- Thevenard syndrome
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.
NIH/National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
60 East 42nd Street
New York, NY 10165
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Center for Peripheral Neuropathy
University of Chicago
5841 South Maryland Ave, MC 2030
Chicago, IL 60637
Foundation for Peripheral Neuropathy
485 Half Day Road
Buffalo Grove, IL 60089
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Last Updated: 7/11/2014
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