논문KCI 등재
A Case of Adult Polyglucosan Body Disease
연세대학교의과대학 (Yonsei University College of Medicine)|2007.08|Yonsei Medical Journal|제48권 제4호|pp. 701-703
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초록
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.
참고문헌 (7)
- Klein CJ, "Adult polyglucosan body disease: case description of an expanding genetic and clinical syndrome", 29, pp.323~328 (2004)
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- Milde P, "Adult polyglucosan body disease", 125, pp.519~522 (2001)
- Robertson NP, "Adult polyglucosan body disease associated with an extrapyramidal syndrome", 65, pp.788~790 (1998)
- Trivedi JR, "Adult polyglucosan body disease associated with lewy bodies and tremor", 60, pp.764~766 (2003)
- Klein CM, "Probable adult polyglucosan body disease", 75, pp.1327~1331 (2000)
- Robitaille Y, "A distinct form of adult polyglucosan body disease with massive involvement of central and peripheral neuronal processes and astrocytes a report of four cases and a review of the occurrence of polyglucosan bodies in other conditions such as Lafora's disease and normal ageing", pp.315~36
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