ISSN:
1432-0533
Keywords:
Type IV Glycogenosis
;
Amylopectinosis
;
Skeletal Muscle
;
Polysaccharides
;
Ultrastructure
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary Skeletal muscles from a patient with type IV glycogenosis were studied by light and electron microscopy. The distinctive polysaccharide deposits were more abundant in the tongue and diaphragm than other skeletal muscles. The involved myofibers contained fusiform areas filled with polyhedral or rounded granules of basophilic material that was further characterized by staining with periodic acid-Schiff, Grocott methenamine silver and alcian blue. Ultrastructurally the deposits were composed of branched filaments, osmophilic granules and electron-dense amorphous material. There are sufficient ultrastructural, histochemical and chemical similarities among the deposits in type IV glycogenosis, Lafora bodies and visceral deposits in myoclonus epilepsy, corpora amylacea and basophilic degeneration of the myocardium to suggest a common composition and mechanism of synthesis possibly through reversal of the debranching enzyme system. However, sharing a biochemical pathway does not necessarily imply a common etiological factor.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00688492
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