adult polyglucosan body disease


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adult polyglucosan body disease

(pŏl″ē-gloo′kŏ-săn)
A rare neurological syndrome that results in peripheral nerve injury, upper motor neuron signs, bowel and bladder dysfunction, and sometimes, familial early-onset dementia. It is caused by the deposition of polyglucosan bodies in skin, central nervous system tissues, peripheral nerves, or sweat glands.
References in periodicals archive ?
The pathogenesis of the adult polyglucosan body disease appears to be heterogeneous.
In summary, adult polyglucosan body disease should be suspected in patients with a late-onset progressive disorder of the peripheral and central nervous system, especially when upper motor neuron signs, dementia, or bladder impairment is present.
A distinct form of adult polyglucosan body disease with massive involvement of central and peripheral neuronal processes and astrocytes: a report of four cases and a review of the occurrence of polyglucosan bodies in other conditions such as Lafora's disease and normal aging.
Adult polyglucosan body disease associated with an extrapyramidal syndrome.
Adult polyglucosan body disease in Ashkenazi Jewish patients carrying the [Tyr.
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