Neuropathy, Hereditary Sensory, Type II

Synonyms

• Congenital Sensory Neuropathy
• Hereditary Sensory and Autonomic Neuropathy, Type II (HSAN Type II)
• HSAN II
• Hereditary Sensory Radicular Neuropathy, Autosomal Recessive
• Radicular Neuropathy, Sensory, Recessive
• Neuropathy, Progressive Sensory, of Children
• Sensory Radicular Neuropathy, Recessive
• Acroosteolysis, Neurogenic
• Acroosteolysis, Giaccai Type
• Morvan Disease

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. Some types of HSN are related to or identical with some forms of Charcot-Marie-Tooth disorder, and others are related to or identical with familial dysautonomia (Riley-Day syndrome). The classification of the HSNs is complicated, and the experts do not always agree on it.

Hereditary sensory neuropathy type II (HSN2) is a rare genetic disorder that usually begins in childhood by affecting the nerves that serve the lower arms and hands and the lower legs and feet (the peripheral nerves). Symptoms start with inflamed fingers or toes especially around the nails. Infection is common and worsens as ulcers (open sores) form on the fingers and on the soles of the feet. The loss of sensation in both hands and feet often leads to neglect of the wounds. This can become serious, even leading to amputation in extreme cases, so it is important to care for any such wounds.

The disorder affects many of the body’s systems, is characterized by early onset (infancy or early childhood) and is transmitted genetically as an autosomal recessive trait.

Resources

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com

National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda, MD 20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Email: braininfo@ninds.nih.gov
Internet: http://www.ninds.nih.gov/

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

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

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.

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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: 8/17/2007
Copyright 1991, 2004, 2007 National Organization for Rare Disorders, Inc.