Polysaccharide (Amylopectin-like) Storage Myopathy Histochemical, Ultrastructural and Biochemical Studies

  • J. F. Pellissier
  • T. de Barsy
  • J. Bille
  • G. Serratrice
  • M. Toga
Conference paper
Part of the Acta Neuropathologica Supplementum book series (NEUROPATHOLOGIC, volume 7)

Summary

A case of an adult polysaccharide myopathy is reported in a patient with progressive muscular atrophy and weakness of limb girdles. Histochemistry and electron microscopy showed in some muscle fibers, a storage material composed of amylopectin-like filaments. Biochemical results were normal and no enzyme deficiency was found. This case is compared with three other published cases. Pathological conditons with amylopectin or amylopectin-like storage material are reviewed.

Key words

myopathy polysaccharide storage glycogenose amylopectin 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Holmes JM, Hougton CR, Woolf AL (1960) A myopathy presenting in adult life with features suggestive of glycogen storage disease. J Neurol Neurosurg Psychiatr 23:302–311PubMedCrossRefGoogle Scholar
  2. 2.
    Karpati G, Carpenter S, Wolfe LS, Sherwin A (1969) A peculiar polysaccharide accumulation in muscle in a case of cardio-skeletalmyopathy. Neurology 19:553–564PubMedGoogle Scholar
  3. 3.
    Radu H, Ionescu V, Georgescu M, Radu A (1974) A new metabolic disorder: Myopathy with glycosamino (sialo) glycans accumulation. Eur Neurol 12:209–225PubMedCrossRefGoogle Scholar
  4. 4.
    Torvik A, Dietrichson P, Svaar H, Hudgson P (1974) Myopathy with tremor and dementia: A metabolic disorder? case report with postmortem study. J Neurol Sci 21:181–190PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 1981

Authors and Affiliations

  • J. F. Pellissier
    • 1
    • 5
  • T. de Barsy
    • 2
  • J. Bille
    • 3
  • G. Serratrice
    • 4
  • M. Toga
    • 1
  1. 1.Laboratoire de NeuropathologieFaculté de MédecineMarseilleFrance
  2. 2.Laboratoire de Chimie Physiologique and International Institute of Cellular and Molecular PathologyBruxellesBelgique
  3. 3.Service de NeurologieHôpital Saint JosephMarseilleFrance
  4. 4.Clinique de Rhumatologie et des Maladies neuromusculairesHôpital de la TimoneMarseilleFrance
  5. 5.Laboratoire de NeuropathologieFaculté de MédecineMarseille Cedex IVFrance

Personalised recommendations