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Preferred term

Hereditary Sensory and Autonomic Neuropathies  

Type

  • mesh:Descriptor

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

Narrower concepts

Entry terms

  • HSAN
  • HSAN (Hereditary Sensory Autonomic Neuropathy)
  • Neuropathies, Hereditary Sensory and Autonomic
  • Sensory and Autonomic Neuropathies, Hereditary

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URI

http://data.loterre.fr/ark:/67375/JVR-VN6PDF3J-5

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