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.
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Resources

Amyotrophic Lateral Sclerosis Association
27001 Agoura Road
Suite 150
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

National Institute of Neurological Disorders and Stroke (NINDS)
P.O. Box 5801
Bethesda, MD 20824
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
TDD: (301)468-5981
Email: me20t@nih.gov
Internet: http://www.ninds.nih.gov/

Rare Cancer Alliance
1649 North Pacana Way
Green Valley, AZ 85614
USA
Tel: (520)625-5495
Fax: (615)526-4921
Email: sharon.lane@rare-cancer.org
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
Email: ordr@od.nih.gov
Internet: http://rarediseases.info.nih.gov/Default.aspx

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

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

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