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Histologisch-histochemische Untersuchungen zur Typologie der Muskelveränderungen bei Atrophia musculorum spinalis pseudomyopathica (Wohlfart—Kugelberg—Welander)

Alterations of muscle with regard to fibre types in atrophia musculorum spinalis pseudomyopathica (wohlfart-kugelberg-welander)

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Summary

In a patient with Wohlfart-Kugelberg-Welander disease a biopsy specimen was taken from the m. quadriceps femoris. Muscle fibres displayed hypertrophy and atrophy of various degrees. Among moderately atrophic fibres type 2 exceeded type 1 significantly in number by 11% compared to the normal distribution. Among extremely atrophic fibres type 2 prevailed even more. Additional dystrophic changes of hypertrophic and atrophic fibres were found more frequently in type 2 than in type 1 fibres. The described alterations point to a mutual pathogenetic dependence of neurogenic and dystrophic lesions of muscle in this disease. In view of the experimentally verified conditions which showed that the motor unit is composed of a single type of fibres only and that in chronic denervating diseases a type-grouping occurs, the disease process on the spinal level can be characterized as one involving type 1 motoneurons in a preferential way. An alternative interpretation of the findings is discussed.

Zusammenfassung

Präparate einer Muskelbiopsie aus dem M. quadriceps femoris eines Patienten mit Wohlfart-Kugelberg-Welanderscher Erkrankung zeigten hypertrophische und neurogen-atrophische Muskelfasern verschiedener Stadien. Verglichen mit der Normalverteilung ließ sich unter den mäßig atrophischen Fasern ein Überwiegen des Typ-2 um 11% gegenüber Typ-1 sichern. Bei den stark atrophischen Fasern lag der Anteil von Typ-2-Fasern noch höher. In hypertrophischen und atrophischen Fasern fanden sich dystrophische Veränderungen, die ebenfalls Typ-2-Fasern bevorzugten. Histochemische und histologische Veränderungen werden i.E. beschrieben und als Zeichen für den Prozeßablauf auf spinaler Ebene diskutiert; dabei ergeben sich Hinweise auf eine pathogenetische Beziehung zwischen neurogenen und myodystrophischen Veränderungen. Unter den experimentell belegten Voraussetzungen vom homogenen Aufbau der motorischen Einheit aus Muskelfasern eines Typs und von der Bildung großer motorischer Einheiten (Typengruppierung) bei chronisch denervierenden Prozessen wird auf eine prädilektive Schädigung von Typ-1-Motoneuronen im Verlauf des spinalen Prozesses geschlossen. Eine alternative Deutung der Befunde wird diskutiert.

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  1. Universitäts-Nervenklinik Marburg a. d. Lahn, Germany

    Rainer Heene

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Heene, R. Histologisch-histochemische Untersuchungen zur Typologie der Muskelveränderungen bei Atrophia musculorum spinalis pseudomyopathica (Wohlfart—Kugelberg—Welander). Z. Neurol. 198, 291–304 (1970). https://doi.org/10.1007/BF00316728

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