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“Megaconial” mitochondria observed in a case of chronic polymyositis

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Summary

Bizarre configurations of muscle mitochondria containing paracrystalline inclusions were demonstrated in a clinically and pathologically typical case of chronic polymyositis with Raynaud's syndrome. The probable mechanism of formation of these abnormal mitochondria was discussed. From the accompanied alterations in the same fibers, i.e. focal assembly of sarcoplasmic reticulum, membranous profiles, miniature mitochondria, and vesicular nuclei with prominent nucleoli, the overall phenomena were interpreted as a process of atypical regeneration.

The detail of the subunits of the paracrystalline inclusions was described and considered to be filamentous structures arranged in double helix. Non-specificity of these mitochondrial abnormalities was evident in 19 other reported cases with a wide range of symptoms, signs, clinical diagnosis, age, and sex distribution. The mitochondrial alterationsper se do not, therefore, reflect any specific muscle disease. Observations of myxovirus-like structures in all 3 biopsy specimens in the present case is discussed in conjunction with the concurrent abnormal mitochondria.

Zusammenfassung

Bizarre Konfiguration von Muskelmitochondrien mit Gehalt an parakristallinen Einschlüssen wurden in einem klinisch-morphologisch typischen Fall von chronischer Polymyositis mit Raynaud-Syndrom beobachtet. Die möglichen Bildungsmechanismen dieser abnormen Mitochondrien werden diskutiert. Aus den Begleitläsionen derselben Fasern, wie fokale Anhäufung von sarkoplasmatischem Reticulum, membranösen Profilen, Miniaturmitochondrien und vesiculären Kernen mit hervortretenden Nucleoli wird das generalisierte Phänomen als atypischer Regenerationsprozeß gedeutet.

Die Details der Untereinheiten der parakristallinen Einschlüsse werden beschrieben und als Fadenstrukturen in Doppel-Helix-Anordnung interpretiert. Die Unspezifität dieser Mitochondrienanomalien ergibt sich aus 19 anderen Fällen des Schrifttums mit einem weiten Spektrum an klinischer Symptomatik, Diagnose, Erkrankungsalter und Geschlechtsverteilung. Die Mitochondrienveränderungan sich ergibt daher keine spezifische Muskelerkrankung.

Der Nachweis von Myxovirus-ähnlichen Strukturen in allen drei Biopsie-Präparaten wird unter Berücksichtigung der gleichzeitig vorliegenden abnormen Mitochondrien diskutiert.

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Authors and Affiliations

  1. Department of Pathology (Neuropathology), University of Wisconsin Medical School, Madison, Wisconsin

    S. M. Chou

  2. Departments of Pathology, Neurosurgery, Neurology (Neuropathology), West Virginia University Medical Center, 26506, Morgantown, West Virginia, USA

    S. M. Chou

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Chou, S.M. “Megaconial” mitochondria observed in a case of chronic polymyositis. Acta Neuropathol 12, 68–89 (1969). https://doi.org/10.1007/BF00685312

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  • DOI: https://doi.org/10.1007/BF00685312

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