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Yonsei University College of Medicine, Yonsei Medical Journal, 4(48), p. 701, 2007

DOI: 10.3349/ymj.2007.48.4.701

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A Case of Adult Polyglucosan Body Disease

Journal article published in 2007 by Seung-Yeob Lee, Jae-Hyeon Park, Se Hun Kim ORCID, Tai-Seung Kim, Won-Joo Kim ORCID, Young-Chul Choi
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

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.