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December 7, 1963JAMAOpen Access

Light and Electron Microscopic Studies of "Myogranules" in Child With Hypotonia and Muscle Weakness

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Authors

PCP.E. ConenLudwig-Maximilians-Universität MünchenEME. Gordon MurphyUniversity of LiverpoolWDW. L. DonohueThe University of Melbourne

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Implication

Case report demonstrates novel rod-shaped myogranules in a child with hypotonia, suggesting an underlying disturbance of muscle proteins.

Key Points

  • Characterize the structural and ultrastructural features of atypical intracellular inclusions found in muscle biopsies from a child with hypotonia and muscle weakness.
  • Screened muscle biopsies from more than 100 individuals using light and electron microscopy.
  • Analyzed paraffin- and plastic-embedded thin sections from two gastrocnemius muscle biopsies taken from a 4-year-old boy presenting with non-progressive hypotonia and weakness.
  • Identified rod-shaped particles designated as myogranules, measuring 0.1 to 5 microns in length and oriented along the fiber long axis, which caused loss of normal cross-striations in affected areas.
  • Observed myofilaments traversing the myogranules with structural periodicity resembling recrystallized muscle protein paramyosin on electron micrographs.

Cite This Study

Conen et al. (1963) studied this question.

synapsesocial.com/papers/6a74fbb7f2cd31a1fadbf191https://doi.org/10.1001/jama.1963.03710100122083
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