Neuropathy, Hereditary Sensory, Type IV

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Neuropathy, Hereditary Sensory, Type IV is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Familial Dysautonomia, Type II
  • Hereditary Sensory and Autonomic Neuropathy IV
  • HSAN IV
  • HSN IV
  • Insensitivity to Pain, Congenital, with Anhydrosis; CIPA
  • Neuropathy, Congenital Sensory, with Anhydrosis

Disorder Subdivisions

  • None

General Discussion

The hereditary sensory neuropathies (HSN) include 4-6 similar but distinct inherited degenerative disorders of the nervous system (neurodegenerative) that frequently progress to loss of feeling, especially in the hands and feet. The classification of these diseases is complicated, and sometimes a source of disagreement among the experts.

Hereditary sensory neuropathy type IV (HSN4) is a rare genetic disorder characterized by the loss of sensation (sensory loss), especially in the feet and legs and, less severely, in the hands and forearms. The sensory loss is due to abnormal functioning of small, unmyelinated nerve fibers and portions of the spinal cord that control responses to pain and temperature as well as other involuntary or automatic body processes. Sweating is almost completely absent with this disorder. Mental retardation is usually present.

The disorder is inherited as an autosomal recessive trait. The gene involved is located on chromosome 1.

HSNs of various types may attack a single nerve (mononeuropathy) or many nerves simultaneously (polyneuropathy). The resulting symptoms may involve sensory, motor, reflex, or blood vessel (vasomotor) functions.

Resources

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Email: ordr@od.nih.gov
Internet: http://rarediseases.info.nih.gov/Default.aspx

Jack Miller Center for Peripheral Neuropathy
University of Chicago
5841 South Maryland Ave, MC 2030
Chicago, IL 60637
Tel: (773)702-5800
Fax: (773)702-5577
Internet: http://millercenter.uchicago.edu

For a Complete Report

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

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