Polyglucosan body disease, adult

Common Name(s)

Polyglucosan body disease, adult

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. 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. Treatment of people with polyglucosan body disease is generally supportive, addressing symptoms such as walking impairment, incontinence, and dementia.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Polyglucosan body disease, adult" for support, advocacy or research.

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Scientific Literature

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Triheptanoin Treatment Trial for Patients With Adult Polyglucosan Body Disease
 

Status: Recruiting

Condition Summary: Adult Polyglucoson Body Disease; Glycogen Brancher Enzyme Deficiency; Glycogen Storage Disease Type IV

 

Last Updated: 13 May 2014

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