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Histochemistry and electron microscopy of muscle fibres in a case of congenital paramyotonia

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Summary

In a case of congenital paramyotonia a muscle biopsy was performed and studied morphologically, histochemically and ultrastructurally. A clearcut pattern of changes has been observed with ATPase and oxidative enzymes. On electron microscopy special changes known as “tubular aggregates” were found. The relationship between the two findings, as well as the significance of such alterations in the range of periodic paralyses and myotonic phenomena, are discussed.

Zusammenfassung

Bei einem Fall von kongenitaler Paramyotonie wurde eine Muskelbiopsie lichtmikroskopisch, histochemisch und elektronenoptisch untersucht. Typische Veränderungen ergaben sich in der ATPase-Färbung und in bezug auf oxydative Enzyme. Bei der elektronenoptischen Untersuchung wurden sogenannte „tubuläre Aggregate“ festgestellt. Es wird die Beziehung der zwei Gruppen von Veränderungen zueinander diskutiert sowie auch die Bedeutung derselben im Rahmen der periodischen Lähmungen und der myotonen Phänomene.

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Schiffer, D., Giordana, M.T., Monga, G. et al. Histochemistry and electron microscopy of muscle fibres in a case of congenital paramyotonia. J. Neurol. 211, 125–133 (1976). https://doi.org/10.1007/BF00313356

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