A Case of Adult Polyglucosan Body Disease.
10.3349/ymj.2007.48.4.701
- Author:
Seung Yeob LEE
1
;
Jae Hyeon PARK
;
So Hun KIM
;
Tai Seung KIM
;
Won Joo KIM
;
Young Chul CHOI
Author Information
1. Department of Neurology, Yonsei University College of Medicine, Seoul, Korea. ycchoi@yuhs.ac
- Publication Type:Case Report
- Keywords:
Adult polyglucosan body disease;
polyglucosan bodies
- MeSH:
Aged;
Biopsy;
*Glucans/metabolism;
Humans;
Inclusion Bodies;
Male;
Neurodegenerative Diseases/metabolism/*pathology
- From:Yonsei Medical Journal
2007;48(4):701-703
- CountryRepublic of Korea
- Language:English
-
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.
