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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Polyglucosan body disease, adult


Other Names for this Disease

  • APBD
  • Polyglucosan body disease, adult form
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

Is fatigue a common symptom of polyglucosan body disease?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

Is fatigue a common symptom of polyglucosan body disease?

Polyglucosan body disease is a progressive neurological disorder that results in upper and lower motor neuron dysfunction. Generalized fatigue can be associated with motor neuron dysfunction as with neurological disorders in general.[1] Fatigue is not an uncommon complaint in people with these disorders.

The following articles provide additional information on this topic. A link to the article abstract is provided below.

Chaudhuri A, Behan PO. Fatigue in neurological disorders. Lancet. 2004 Mar 20;363(9413):978-88.

Krupp LB, Pollina DA. Mechanisms and management of fatigue in progressive neurological disorders. Curr Opin Neurol. 1996 Dec;9(6):456-60.

To obtain the full article, contact a medical/university library (or your local library for interlibrary loan), or order it online using the links above. The National Library of Medicine (NLM) Web site has a page for locating libraries in your area that can provide direct access to these journals (print or online). The Web page also describes how you can get these articles through interlibrary loan and Loansome Doc (an NLM document-ordering service). You can access this page at the following link http://nnlm.gov/members/. You can also contact the NLM toll-free at 888-346-3656 to locate libraries in your area.

Last updated: 12/2/2008

What is polyglucosan body disease?

Polyglucosan body disease is a slowly progressive metabolic disorder. It is caused by excessive accumulation of polyglucosan bodies in tissues, including nerve, muscle, liver, kidney, and lung. The disease can cause neurogenic bladder, dementia, loss of feeling in the lower limbs, and upper and lower motor neuron dysfunction.[2] A variety of different biochemical defects may cause polyglucosan body disease. Glycogen branching enzyme (GBE) deficiency has been identified as the cause in some patients.[3] Treatment of people with polyglucosan body disease is generally supportive, addressing symptoms such as walking impairment, incontinence, and dementia.[2]
Last updated: 12/2/2008

References
  • Krivickas LS. Motor Neuron Disease. In: Frontera WR Silver JK Rizzo TD. Essentials of Physical Medicine and Rehabilitation, 2nd ed. Philadelpia PA: Saunders; 2008;
  • Klein CM. Adult Polyglucosan Body Disease. NORD Guide to Rare Disorders. 2003;
  • Moses SW, Parvari R. The variable presentations of glycogen storage disease type IV: a review of clinical, enzymatic, and molecular studies. Curr Mol Med. 2002 Mar;
Other Names for this Disease
  • APBD
  • Polyglucosan body disease, adult form
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.