title: A case of adult polyglucosan body disease.
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.
authors.affiliation: Department of Neurology, Yongdong Severance Hospital, Yonsei University College of Medicine, 146-92 Dogok-dong, Gangnam-gu, Seoul 135-720, Korea.
authors.forename: Seung-Yeob
authors.initials: SY
authors.lastname: Lee
authors.forename: Jae-Hyeon
authors.initials: JH
authors.lastname: Park
authors.forename: So Hun
authors.initials: SH
authors.lastname: Kim
authors.forename: Tai-Seung
authors.initials: TS
authors.lastname: Kim
authors.forename: Won-Joo
authors.initials: WJ
authors.lastname: Kim
authors.forename: Young-Chul
authors.initials: YC
authors.lastname: Choi
mesh_terms: D000368:Aged; D001706:Biopsy; D005936:Glucans / Q000378:metabolism; D006801:Humans; D002479:Inclusion Bodies; D008297:Male; D019636:Neurodegenerative Diseases / Q000378:metabolism / Q000473:pathology*
chemical_list: D005936:Glucans; C083094:polyglucosan