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Hereditary Sensory and Autonomic Neuropathy Type II

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Hereditary Sensory and Autonomic Neuropathy Type II 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

  • HSAN II
  • Acroosteolysis, Neurogenic
  • Acroosteolysis, Giaccai Type
  • Morvan Disease
  • Hereditary Sensory Neuropathy Type II
  • HSAN2

Disorder Subdivisions

  • None

General Discussion

Summary
Hereditary sensory and autonomic neuropathy type II (HSAN2) is a rare genetic disorder that usually begins in childhood by affecting the nerves that serve the lower legs and feet and the lower arms and hands. Symptoms start with inflamed fingers or toes, especially around the nails. Numbness and tingling sensations in the hands and feet may also occur. Eventually, affected individuals lose feeling (sensation) in the hands and feet. This sensory loss is due to abnormal functioning of the sensory nerves that control responses to pain and temperature and may also affect the autonomic nervous system that controls other involuntary or automatic body processes. Chronic infection of the affected areas is common and worsens as ulcers form on the fingers or the soles of the hands and feet. The loss of sensation in the hands and feet often leads to neglect of the wounds. This can become serious even leading to amputation in extreme cases if left untreated. The disorder affects many of the body's systems, is characterized by early onset (infancy or childhood) and is transmitted genetically as an autosomal recessive trait. HSAN2 occurs due to mutations in specific genes. There are a few subtypes designated A through C, each one associated with a different gene.

Introduction
The hereditary sensory and autonomic neuropathies (HSAN), also known as the hereditary sensory neuropathies, include at least six similar but distinct inherited degenerative disorders of the nervous system (neurodegenerative) that frequently progress to loss of feeling, especially in the hands and feet. Some of these disorders have several subtypes based upon the specific associated genes. Some types of HSAN are related to or identical with some forms of Charcot-Marie-Tooth disease, and others are related to or identical with familial dysautonomia (Riley-Day syndrome). The classification of the HSANs is complicated, and the experts to not always agree on it. Furthermore, HSANs are classified as broadly as peripheral neuropathies or disorders or the peripheral nervous system, which encompasses all of the nerves outside of the central nervous system (i.e. brain and spinal cord).

Resources

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)997-4488
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com

NIH/National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
TDD: (301)468-5981
Internet: http://www.ninds.nih.gov/

Neuropathy Association
60 East 42nd Street
Suite 942
New York, NY 10165
USA
Tel: (212)692-0662
Fax: (212)692-0668
Email: info@neuropathy.org
Internet: http://www.neuropathy.org

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
Internet: http://rarediseases.info.nih.gov/GARD/

Center for Peripheral Neuropathy
University of Chicago
5841 South Maryland Ave, MC 2030
Chicago, IL 60637
Tel: (773)702-5659
Fax: (773)702-5577
Internet: http://peripheralneuropathycenter.uchicago.edu/

Foundation for Peripheral Neuropathy
485 Half Day Road
Suite 200
Buffalo Grove, IL 60089
Tel: (847)883-9942
Fax: (847)883-9960
Tel: (877)833-9942
Email: info@tffpn.org
Internet: http://www.foundationforpn.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  7/17/2013
Copyright  1991, 2004, 2013 National Organization for Rare Disorders, Inc.

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