Polyglucosan Body Disease, Adult
National Organization for Rare Disorders, Inc.
- Polyglucosan Body Disease, Adult Form
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.
Amyotrophic Lateral Sclerosis Association
27001 Agoura Road
Calabasas Hills, CA 91301-5104
NIH/National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
Rare Cancer Alliance
1649 North Pacana Way
Green Valley, AZ 85614
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Adult Polyglucosan Body Disease Research Foundation
8 West 37th Street, Suite 901
New York, NY 10018
Frederick R Corne Foundation
PO Box 1276
Perrysburg, OH 43552-1246
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Last Updated: 2/5/2008
Copyright 1995, 2004 National Organization for Rare Disorders, Inc.
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