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    Hereditary Sensory and Autonomic Neuropathy Type IV

    Important
    It is possible that the main title of the report Hereditary Sensory and Autonomic Neuropathy 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 Neuropathy Type IV
    • HSAN IV
    • HSNAN4
    • Congenital Insensitivity to Pain with Anhidrosis (CIPA)

    Disorder Subdivisions

    • None

    General Discussion

    Summary
    Hereditary sensory and autonomic neuropathy type IV (HSAN4 or HSAN IV) is a rare genetic disorder that usually begins in infancy and is characterized by an inability to feel pain and an inability to sweat (anhidrosis). Affected individuals also cannot feel temperature and cannot distinguish between hot and cold. The sensory loss in individuals with HSAN IV is due to abnormal functioning of the sensory nerves that control responses to pain and temperature. Anhidrosis can cause recurrent episodes of fever and high body temperature. An inability to feel pain can lead to unintentional self-mutation, repeated fractures, and joint damage. Affected individuals and especially children or infants may be unaware of injury delaying treatment. HSAN IV is caused by mutations in the NTRK1 gene. The disorder is inherited in an autosomal recessive manner.

    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 more 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). HSAN IV is also known as congenital insensitivity to pain with anhidrosis (CIPA).

    Resources

    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: 5/15/2014
    Copyright 2004, 2007, 2014 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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