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