Diseases

Hereditary Sensory and Autonomic Neuropathies

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. (From Joynt, Clinical Neurology, 1995, Ch51, pp142-4)

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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. (From Joynt, Clinical Neurology, 1995, Ch51, pp142-4)

MeSH Record

Classification

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MeSH Record

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MeSH Record

Synonyms

76 entry terms
  • HSAN
  • HSAN (Hereditary Sensory Autonomic Neuropathy)
  • Neuropathies, Hereditary Sensory and Autonomic
  • Sensory and Autonomic Neuropathies, Hereditary
  • HSANs (Hereditary Sensory Autonomic Neuropathy)
  • Acroosteolysis, Giaccai Type
  • Acroosteolysis, Neurogenic
  • Congenital Insensitivity to Pain with Anhidrosis
  • Familial Dysautonomia, Type 2
  • Familial Dysautonomia, Type II
  • Giaccai Type Acroosteolysis
  • HSAN 1
  • HSAN 4
  • HSAN 5
  • HSAN I
  • HSAN IV
  • HSAN Type I
  • HSAN Type II
  • HSAN Type IV
  • HSAN Type V
  • HSAN V
  • HSAN2
  • HSAN5
  • HSANII
  • HSN Type I
  • HSN Type II
  • Hereditary Sensory And Autonomic Neuropathy IV
  • Hereditary Sensory Autonomic Neuropathy, Type 1
  • Hereditary Sensory Autonomic Neuropathy, Type 2
  • Hereditary Sensory Autonomic Neuropathy, Type 4
  • Hereditary Sensory Autonomic Neuropathy, Type 5
  • Hereditary Sensory Neuropathy Type 1
  • Hereditary Sensory Neuropathy Type I
  • Hereditary Sensory Neuropathy Type Ia
  • Hereditary Sensory Radicular Neuropathy
  • Hereditary Sensory Radicular Neuropathy, Recessive Form
  • Hereditary Sensory and Autonomic Neuropathy 4
  • Hereditary Sensory and Autonomic Neuropathy Type 1
  • Hereditary Sensory and Autonomic Neuropathy Type 2
  • Hereditary Sensory and Autonomic Neuropathy Type I
  • Hereditary Sensory and Autonomic Neuropathy Type II
  • Hereditary Sensory and Autonomic Neuropathy Type IV
  • Hereditary Sensory and Autonomic Neuropathy Type V
  • Hereditary Sensory and Autonomic Neuropathy, Type 4
  • Hereditary Sensory and Autonomic Neuropathy, Type 5
  • Insensitivity to Pain with Anhidrosis, Congenital
  • Insensitivity to Pain, Congenital, with Anhidrosis
  • Neurogenic Acroosteolysis
  • Neuropathy Hereditary Sensory Radicular, Autosomal Dominant
  • Neuropathy Hereditary Sensory and Autonomic Type 1
  • Neuropathy, Congenital Sensory
  • Neuropathy, Congenital Sensory, with Anhidrosis
  • Neuropathy, Hereditary Sensory And Autonomic, Type I
  • Neuropathy, Hereditary Sensory And Autonomic, Type V
  • Neuropathy, Hereditary Sensory Radicular, Autosomal Dominant
  • Neuropathy, Hereditary Sensory Radicular, Autosomal Recessive
  • Neuropathy, Hereditary Sensory, Type I
  • Neuropathy, Progressive Sensory, Of Children
  • Pain Insensitivity with Anhidrosis, Congenital
  • Sensory Neuropathy, Hereditary
  • Acroosteolyses, Neurogenic
  • Congenital Sensory Neuropathies
  • Congenital Sensory Neuropathy
  • HSN Type IIs
  • Hereditary Sensory Neuropathies
  • Hereditary Sensory Neuropathy
  • Neurogenic Acroosteolyses
  • Neuropathies, Congenital Sensory
  • Neuropathies, Hereditary Sensory
  • Neuropathy, Hereditary Sensory
  • Sensory Neuropathies, Congenital
  • Sensory Neuropathies, Hereditary
  • Sensory Neuropathy, Congenital
  • Type I, HSAN
  • Type I, HSN
  • Type IV, HSAN

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

Indexing Annotation

do not confuse with HEREDITARY SENSORY AND MOTOR NEUROPATHY; note entry terms for HSAN types: HSAN TYPE III see DYSAUTONOMIA, FAMILIAL is also available

MeSH Record

History Note

2000(1989); use NEUROPATHY, HEREDITARY SENSORY 1979-1988

MeSH Record

Previous Indexing

  • Peripheral Nerve Diseases (1966-1978)
  • Peripheral Nerves (1966-1978)
  • Sensation (1966-1978)

MeSH Hierarchy

Tree Numbers

  1. C10w.500w.250w
  2. C10w.574w.500.493w
  3. C10w.668w.829w.800w.175w
  4. C16w.131w.666w.310w
  5. C16w.320w.400.415w

AMA Style

References

  1. National Library of Medicine. Hereditary Sensory and Autonomic Neuropathies. Medical Subject Headings (MeSH). 2026. Unique ID D009477. http://id.nlm.nih.gov/mesh/2026/D009477
  2. Hereditary Sensory and Autonomic Neuropathies. In: Wikipedia. https://en.wikipedia.org/wiki/Hereditary_sensory_and_autonomic_neuropathy
  3. Hereditary Sensory and Autonomic Neuropathies. In: Wikidata. https://www.wikidata.org/wiki/Q3702898