Summary
Bunina inclusion bodies were distributed abundantly in the nerve cell cytoplasm of a case of amyotrophic lateral sclerosis (ALS). They appeared mainly in the motor nuclei of the brainstem and spinal cord on a routine examination. Although the oculomotor nucleus is known to be spared in ALS, Bunina bodies were found in 17% of the cells when examined in serial sections, much fewer than were found in other motor nuclei of the brainstem. Bunina bodies were also found in 7.7% of the nerve cells of Onuf's nucleus in the ventral part of the anterior horn of the sacral cord, which has been thought to innervate the striated muscles of the vesicorectal sphincters and also to be spared in ALS. The number was less than in the lateral part of the anterior horn but the same as in the intermediolateral nucleus. This selective appearance of Bunina bodies in ALS may indicate that this inclusion is related to the primary functional disturbance in the motor neurons of ALS.
Zusammenfassung
Es wurde die Verteilung der Bunina-Körperchen bei einem Fall der amyotrophischen Lateralsklerose (ALS) untersucht, der diese Einschlußkörperchen im Zytoplasma der vielen Nervenzellen gezeigt hat. Bei gewöhnlichen Untersuchungen waren die Bunina-Körperchen hauptsächlich in motorischen Kernen des Hirnstamms und Rückenmarks aufgetreten. Im Okulomotorkern, der bei ALS gut erhalten ist, wurden die Bunina-Körperchen in 17% Nervenzellen bei Serienschnitten gefunden, d. h. sie zeigen sich viel weniger zahlreich als in anderen Hirnstamm-Motorkernen. Im Sakralsegment des Rückenmarks, nämlich im Onufschen Kern im Vorderhorn, der die gestreifte Muskulatur der Vesikorektalsphinkter innerviert und bei ALS nicht gestört ist, zeigten sich die Bunina-Körperchen in 7.7% der Nervenzellen. Die Häufigkeit ist geringer als die im Lateralteil des Vorderhorns (postero-postero-lateral) und ungefähr gleich mit den im Intermediolateralkern.
Es wird angenommen, daß dieses elektive Auftreten der Bunina-Körperchen zeigt, daß die Einschlußkörperchen in Beziehung zu einer primären Veränderung und Funktionsstörung der motorischen Neuronen bei der ALS stehen.
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Tomonaga, M. Selective appearance of Bunina bodies in amyotrophic lateral sclerosis. J. Neurol. 223, 259–267 (1980). https://doi.org/10.1007/BF00313340
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DOI: https://doi.org/10.1007/BF00313340