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Polyglucosan Body Disease, Adult

Important
It is possible that the main title of the report Polyglucosan Body Disease, Adult 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

APBD
Polyglucosan Body Disease, Adult Form

Disorder Subdivisions

None

General Discussion

Adult polyglucosan body disease (APBD) is a rare, chronically progressive, metabolic disorder with severe neurological expression. It is caused by the abnormal accumulation of microscopic material (polyglucosan bodies), predominantly within the myelinated nerve fibers (motor neurons). The polyglucosan bodies are spherical and composed of large, complex, sugar-based molecules (branched polysaccharides).

The disorder typically affects both upper and lower motor neurons, resulting from nerve damage within the brain and spinal cord (central nervous system) respectively. Symptoms usually begin during middle age or later and usually include muscle weakness, loss of sensation, and/or wasting of muscles (atrophy) in the arms and/or legs. Impaired bladder control (neurogenic bladder) and/or mental confusion (dementia) also occur.
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Resources

Amyotrophic Lateral Sclerosis Association
27001 Agoura Road
Suite 150
Calabasas Hills, CA 91301-5104
USA
Tel: 8188809007
Fax: 8188809006
Tel: 8007824747
TDD: 8185933540
Email: als@alsa-national.org
Internet: http://www.alsa.org

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/

APBD Research Foundation
8 West 37th Street
R.901
New York, NY 10018
Tel: (212)643-1221
Fax: (212)643-0963
Email: info@APBDRF.org
Internet: http://www.apbdrf.org

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.

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: 2/5/2008
Copyright 1995, 2004 National Organization for Rare Disorders, Inc.

WebMD Medical Reference from the National Organization of Rare Disorders

Last Updated: February 05, 2008

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.