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Histochemical and electron microscopic observations on “onion bulb” formations in a case of hypertrophic neuritis of 25 years duration with onset in childhood

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Summary

Histochemical and electron microscopic data are presented from a patient with hypertrophic neuritis of 25 years duration with onset in childhood. The present observations and those of recent investigators form the basis of a discussion of various pathogenetic hypotheses for “onion bulb” structures in the nerves from patients with hypertrophic neuritis. It is concluded that the onion bulb lesion is a nonspecific manifestation of chronic segmental demyelinization resulting from a primary or acquired abnormality of Schwann cells.

Zusammenfassung

Histochemische und elektronenoptische Befunde eines Patienten mit hypertrophischer Neuritis von 25 Jahren Dauer mit Beginn der Erkrankung in der Kindheit werden mitgeteilt. Die erhobenen Befunde sowie andere jüngste Beobachtungen bilden die Grundlage einer Diskussion der verschiedenen pathogenetischen Hypothesen der “Zwiebelschalen”-Strukturen in den Nerven von Patienten mit hypertrophischer Neuritis. Die Zwiebelschalenbildung wird als unspezifische Manifestation einer chronischen segmentalen Entmarkung infolge primärer oder erworbener Anomalie der Schwann-Zellen interpretiert.

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Authors and Affiliations

  1. Ayer Clinical Laboratory and Department of Neurology, The Pennsylvania Hospital, Philadelphia, Pa., USA

    S. I. Zacks (Assoc. Prof. of Pathology), H. Lipshutz & F. Elliott

  2. School of Medicine Pennsylvania Hospital Department for Sick and Injured, University of Pennsylvania, Eight and Spruce Streets, 19107, Philadelphia, Pa., USA

    S. I. Zacks (Assoc. Prof. of Pathology)

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  1. S. I. Zacks
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  2. H. Lipshutz
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  3. F. Elliott
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Zacks, S.I., Lipshutz, H. & Elliott, F. Histochemical and electron microscopic observations on “onion bulb” formations in a case of hypertrophic neuritis of 25 years duration with onset in childhood. Acta Neuropathol 11, 157–173 (1968). https://doi.org/10.1007/BF00690218

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