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)

Also Known As:

HSAN; Congenital Insensitivity to Pain with Anhidrosis; Hereditary Sensory Neuropathy; Neuropathy, Hereditary Sensory; Insensitivity to Pain with Anhidrosis, Congenital; Acroosteolysis, Giaccai Type; Acroosteolysis, Neurogenic; Familial Dysautonomia, Type 2; Familial Dysautonomia, Type II; Giaccai Type Acroosteolysis; HSAN (Hereditary Sensory Autonomic Neuropathy); HSAN 1; HSAN 4; HSAN 5; HSAN I; HSAN IV; HSAN V; HSAN2; HSAN5; HSANII; 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, 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; Acroosteolyses, Neurogenic; Congenital Sensory Neuropathies; Congenital Sensory Neuropathy; HSANs (Hereditary Sensory Autonomic Neuropathy); HSN Type IIs; Hereditary Sensory Neuropathies; Neurogenic Acroosteolyses; Neuropathies, Congenital Sensory; Neuropathies, Hereditary Sensory; Sensory Neuropathies, Congenital; Sensory Neuropathies, Hereditary; Sensory Neuropathy, Congenital; Type I, HSAN; Type I, HSN; Type IV, HSAN; HSAN Type I; HSAN Type II; HSAN Type IV; HSAN Type V; HSN Type I; HSN Type II; Neuropathies, Hereditary Sensory and Autonomic; Pain Insensitivity with Anhidrosis, Congenital; Sensory Neuropathy, Hereditary; Sensory and Autonomic Neuropathies, Hereditary

Nervous System Diseases: 14737
- Neurodegenerative Diseases: 44095
  - Nervous System Heredodegenerative Disorders: 62
    - Hereditary Sensory and Autonomic Neuropathies: 332
- Neuromuscular Diseases: 2048
  - Peripheral Nervous System Diseases: 11768
    - Polyneuropathies: 5219
      - Hereditary Sensory and Autonomic Neuropathies: 332
- Nervous System Malformations: 36
  - Hereditary Sensory and Autonomic Neuropathies: 332
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 960
- Congenital Abnormalities: 26404
  - Nervous System Malformations: 36
    - Hereditary Sensory and Autonomic Neuropathies: 332
- Inborn Genetic Diseases: 12342
  - Nervous System Heredodegenerative Disorders: 62
    - Hereditary Sensory and Autonomic Neuropathies: 332

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