eISSN: 1509-572x
ISSN: 1641-4640
Folia Neuropathologica
Current issue Archive Manuscripts accepted About the journal Editorial board Journal's reviewers Abstracting and indexing Subscription Contact Instructions for authors Ethical standards and procedures
SCImago Journal & Country Rank
 
3/2008
vol. 46
 
Share:
Share:
more
 
 
abstract:

Immunohistochemical and ultrastructural changes in the brain in probable adult glycogenosis type IV: adult polyglucosan body disease

Teresa Wierzba-Bobrowicz
,
Eliza Lewandowska
,
Tomasz Stępień
,
Joanna Modzelewska

Folia Neuropathol 2008; 46 (3): 165-175
Online publish date: 2008/09/19
View full text
Get citation
ENW
EndNote
BIB
JabRef, Mendeley
RIS
Papers, Reference Manager, RefWorks, Zotero
AMA
APA
Chicago
Harvard
MLA
Vancouver
 
Glycogenosis type IV is caused by a deficiency of glycogen branching enzyme (α-1,4 glucan 6-transglucosylase). Adult polyglucosan body disease (APBD) may represent a neuropathological hallmark of the adult form of this storage disease of the central nervous system.
We analysed a case of a 45-year-old unconscious woman who died three days after admission to the hospital.
Neuropathological examination revealed massive accumulation of polyglucosan bodies (PBs) in the cortex and white matter of the whole brain. PBs were located in the processes of neurons, astrocytes and microglial cells. The storage material in the cytoplasm of neurons and glial cells was visible as fine granules.
Ultrastructurally, PBs consisted of non-membrane-bound deposits of branched and densely packed filaments, measuring about 7–10 nm in diameter, typical of polyglucosan bodies.
APBD patients develop upper and lower neuron disease and dementia, probably secondary to the disruption of neuron and astrocyte functions.
keywords:

adult polyglucosan body disease, glycogenosis type IV, ultrastructure

Quick links
© 2021 Termedia Sp. z o.o. All rights reserved.
Developed by Bentus.