Document Detail

On the association of excess glycogen granules and polyglucosan bodies (corpora amylacea) in astrocytes of a 17-year-old patient with a neurologic disease of unknown origin: clinical, biochemical, and ultrastructural observations.
MedLine Citation:
PMID:  7166016     Owner:  NLM     Status:  MEDLINE    
The case of a 17-year-old patient with a severe progressive neurologic disease is presented. Enzymatic studies revealed that total activity of both hexosaminidases A and B against the synthetic substrate was markedly reduced and that hexosaminidase B activity, studied by heat inactivation and isoelectric focusing, was even more reduced. Rectum and skin biopsies studied by electron microscopy were non-contributory. Brain biopsy was histologically normal. With electron microscopy many filamentous bodies were observed mainly in astrocytic processes; they were interpretable as intra-astrocytic polyglucosan bodies (the corpora amylacea variety of polyglucosan bodies). In addition, an excess of non-membrane-bound glycogen particles, mostly within astrocytes, was also present. Some degree of gliosis was noticed, as well as thickening and redundancy of the basement membrane of capillary vessels. The case is discussed and compared with the conditions described in the literature where either accumulation of glycogen or formation of polyglucosan bodies (corpora amylacea, Lafora-like bodies, Lafora bodies, Bielschowsky bodies, amylopectin bodies), or both occur. Since none of them, however, adequately fit the present case, it must remain undiagnosed.
L Palmucci; A P Anzil; H Christomanou
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical neuropathology     Volume:  1     ISSN:  0722-5091     ISO Abbreviation:  Clin. Neuropathol.     Publication Date:  1982  
Date Detail:
Created Date:  1983-05-27     Completed Date:  1983-05-27     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8214420     Medline TA:  Clin Neuropathol     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  2-10     Citation Subset:  IM    
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MeSH Terms
Amylopectin / metabolism*
Astrocytes / ultrastructure*
Brain Diseases, Metabolic / pathology*
Cerebral Cortex / pathology*
Cytoplasmic Granules / metabolism*
Glycogen / metabolism*
Hexosaminidases / deficiency
Microscopy, Electron
Reg. No./Substance:
9005-79-2/Glycogen; 9037-22-3/Amylopectin; EC 3.2.1.-/Hexosaminidases

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