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Polyglucosan Body Disease, Adult

National Organization for Rare Disorders, Inc.

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.

Resources

Amyotrophic Lateral Sclerosis Association

27001 Agoura Road

Suite 250

Calabasas Hills, CA 91301-5104

USA

Tel: (818)880-9007

Fax: (818)880-9006

Tel: (800)782-4747

TDD: (818)593-3540

Email: als@alsa-national.org

Internet: http://www.alsa.org



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/



Rare Cancer Alliance

1649 North Pacana Way

Green Valley, AZ 85614

USA

Internet: http://www.rare-cancer.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/



Adult Polyglucosan Body Disease Research Foundation

8 West 37th Street, Suite 901

New York, NY 10018

Tel: (646)580-5610

Fax: (212)643-0963

Email: info@APBDRF.org

Internet: http://www.apbdrf.org



Frederick R Corne Foundation

PO Box 1276

Perrysburg, OH 43552-1246

Tel: (419)407-6425

Email: frcfound@frcfound.org

Internet: http://www.frcfound.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 MyD-H, the Dartmouth-Hitchcock patient portal. You must be a registered MyD-H user for the Lebanon, Manchester, or Nashua locations to access this site.

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.

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