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KMID : 0311120070480040701
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Yonsei Medical Journal
2007 Volume.48 No. 4 p.701 ~ p.703
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A Case of Adult Polyglucosan Body Disease
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Lee Seung-Yeob
Kim Tai-Seung
Park Jae-Hyun
Kim Won-Joo
Choi Young-Chul
Kim Se-Hoon
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Abstract
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Adult polyglucosan body disease (APBD) is a rare neurological disease, characterized by adult onset (fifth to seventh decades), progressive sensorimotor or pure motor peripheral neuropathy, upper motor neuron symptoms, neurogenic bladder, and cognitive impairment. APBD is confirmed by a sural nerve biopsy that shows the widespread presence of polyglucosan bodies in the nerve. We report a 70 year old male patient who exhibited progressive weakness in all extremities and dementia. His electrodiagnostic studies showed sensorimotor polyneuropathy and muscle pathology that consisted of polyglucosan bodies located in small peripheral nerves. This is the first case of APBD reported in Korea.
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KEYWORD
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Adult polyglucosan body disease, polyglucosan bodies
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