8C21.1

ICD-11 MMS 2026-01

Hereditary sensory and autonomic neuropathy type III

Description

Hereditary sensory and autonomic neuropathy, type 3 (HSAN3) is an autosomal recessive disorder seen primarily in Ashkenazi Jewish children caused by a mutation in the I-kappa B kinase associated protein. It is characterised by sensory dysfunction and severe impairment of the autonomic nervous system activity, resulting in multisystem dysfunction. Symptoms can include insensitivity to pain and temperature, intact visceral pain, alacrima, hypoactive corneal and tendon reflexes and absence of lingual fungiform papillae.

Index terms

  • Hereditary sensory and autonomic neuropathy type III
  • Familial dysautonomia
  • HSAN3 - [Hereditary sensory and autonomic neuropathy type 3]
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  • Riley Day syndrome

Exclusions from above levels

  • FB56 – neuritis NOS
  • http://id.who.int/icd/entity/114997967 – Injury of nerves or lumbar spinal cord at abdomen, lower back or pelvis level
  • http://id.who.int/icd/entity/1454363907 – Injury of nerves or spinal cord at neck level
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  • NA04 – Injury of cranial nerves
  • NC14 – Injury of nerves at shoulder or upper arm level
  • NC34 – Injury of nerves at forearm level
  • NC55 – Injury of nerves at wrist or hand level
  • NC74 – Injury of nerves at hip or thigh level
  • NC94 – Injury of nerves at lower leg level
  • ND15 – Injury of nerves at ankle or foot level
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8C21.0 – Hereditary sensory and autonomic neuropathy type I
  • 8C21.2 – Hereditary sensory and autonomic neuropathy type IV
  • 8C21.3 – Hereditary sensory and autonomic neuropathy type V
  • 8C21.Y – Other specified hereditary sensory or autonomic neuropathy
  • 8C21.Z – Hereditary sensory or autonomic neuropathy, unspecified

Foundation entity

Hereditary sensory and autonomic neuropathy type III – http://id.who.int/icd/entity/831377479

Part of: 8C21: Hereditary sensory or autonomic neuropathy › 08: Diseases of the nervous system