Preferred Name

Hereditary Sensory and Autonomic Neuropathies
Synonyms

Hereditary Sensory Radicular Neuropathy

Congenital Insensitivity to Pain with Anhidrosis

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).

ID

http://uri.neuinfo.org/nif/nifstd/birnlex_12556

acronym

HSAN

alternative label

Hereditary Sensory Radicular Neuropathy

Congenital Insensitivity to Pain with Anhidrosis

createdDate

2007-10-05

definingCitation

Joynt, Clinical Neurology, 1995, Ch51, pp142-4

definingCitationURI

http://www.ninds.nih.gov/disorders/neuropathy_hereditary/neuropathy_hereditary.htm

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).

editorial note

http://uri.neuinfo.org/nif/nifstd/readable/uncurated

externalSourceId

D009477

hasBirnlexCurator

http://uri.neuinfo.org/nif/nifstd/readable/Bill_Bug

hasCurationStatus

http://uri.neuinfo.org/nif/nifstd/readable/uncurated

hasExternalSource

http://uri.neuinfo.org/nif/nifstd/readable/MeSH

label

Hereditary Sensory and Autonomic Neuropathies

MeshUid

D009477

modifiedDate

2007-10-05

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).

http://uri.neuinfo.org/nif/nifstd/readable/uncurated

preferred label

Hereditary Sensory and Autonomic Neuropathies

Resource Identifier

D009477

scope note

Do not confuse with HEREDITARY MOTOR AND SENSORY NEUROPATHIES; for X refs to HSAN types: HSAN TYPE III is see DYSAUTONOMIA, FAMILIAL.

synonym

Hereditary Sensory Radicular Neuropathy

Congenital Insensitivity to Pain with Anhidrosis

subClassOf

http://purl.obolibrary.org/obo/DOID_4308

http://uri.neuinfo.org/nif/nifstd/birnlex_12553

http://purl.obolibrary.org/obo/DOID_1389

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