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Neuroscience Information Framework (NIF) Dysfunction Ontlogy
Last uploaded:
April 11, 2018
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| Preferred Name | Hereditary Sensory and Autonomic Neuropathies | |
| Synonyms |
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| Definitions |
A group of inherited disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and clinically by loss of sensation and autonomic dysfunction. There are five subtypes. Type I features autosomal dominant inheritance and distal sensory involvement. Type II is characterized by autosomal inheritance and distal and proximal sensory loss. Type III is DYSAUTONOMIA, FAMILIAL. Type IV features insensitivity to pain, heat intolerance, and mental deficiency. Type V is characterized by a selective loss of pain with intact light touch and vibratory sensation (MeSH). |
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| ID |
http://uri.neuinfo.org/nif/nifstd/birnlex_12556 |
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| acronym |
HSAN
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| createdDate |
2007-10-05
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| definingCitation |
Joynt, Clinical Neurology, 1995, Ch51, pp142-4
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| definingCitationURI |
http://www.ninds.nih.gov/disorders/neuropathy_hereditary/neuropathy_hereditary.htm |
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| definition |
A group of inherited disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and clinically by loss of sensation and autonomic dysfunction. There are five subtypes. Type I features autosomal dominant inheritance and distal sensory involvement. Type II is characterized by autosomal inheritance and distal and proximal sensory loss. Type III is DYSAUTONOMIA, FAMILIAL. Type IV features insensitivity to pain, heat intolerance, and mental deficiency. Type V is characterized by a selective loss of pain with intact light touch and vibratory sensation (MeSH).
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| editorial note | ||
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| hasCurationStatus | ||
| hasExternalSource | ||
| label |
Hereditary Sensory and Autonomic Neuropathies
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| MeshUid |
D009477
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| modifiedDate |
2007-10-05
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| note |
Do not confuse with HEREDITARY MOTOR AND SENSORY NEUROPATHIES; for X refs to HSAN types: HSAN TYPE III is see DYSAUTONOMIA, FAMILIAL. A group of inherited disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and clinically by loss of sensation and autonomic dysfunction. There are five subtypes. Type I features autosomal dominant inheritance and distal sensory involvement. Type II is characterized by autosomal inheritance and distal and proximal sensory loss. Type III is DYSAUTONOMIA, FAMILIAL. Type IV features insensitivity to pain, heat intolerance, and mental deficiency. Type V is characterized by a selective loss of pain with intact light touch and vibratory sensation (MeSH).
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| preferred label |
Hereditary Sensory and Autonomic Neuropathies
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| scope note |
Do not confuse with HEREDITARY MOTOR AND SENSORY NEUROPATHIES; for X refs to HSAN types: HSAN TYPE III is see DYSAUTONOMIA, FAMILIAL.
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| synonym |
Hereditary Sensory Radicular Neuropathy Congenital Insensitivity to Pain with Anhidrosis
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