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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

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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References

  1. Achard, E., etJ. Thiers: Polynévrite Chronique Hypertrophique de l'adulte. Rev. neurol.3, 146–150 (1924).

  2. Aitkin, J. T., M. Sharman, andJ. Z. Young: Maturation of regenerating nerve fibers with various peripheral connexions. J. Anat. (Lond.)81, 1–22 (1947).

  3. Austin, J. H.: Observations on the syndrome of hypertrophic neuritis. (The hypertrophic interstitial radiculo-neuropathies). Medicine (Baltimore)35, 187–239 (1956).

  4. Bielschowsky, M.: Familiäre hypertrophische Neuritis und Neurofibromatose. J. Psychol. Neurol. (Lpz.)24, 182–205 (1922).

  5. Boveri, P.: De la Névrite Hypertrophique Familiale. Sem. méd. (Paris)30, 145–150 (1910).

  6. Cammermeyer, J.: Neuropathologic changes in hereditary neuropathies: Manifestations of the syndrome heredopathia atactica polyneuritiformes in the presence of interstitial hypertrophic polyneuropathy. J. Neuropath. exp. Neurol.15, 340–361 (1956).

  7. Dalton, A. J.: A chrome-osmium fixative for electron microscopy. Anat. Rec.121, 281 (1955).

  8. DeBruyn, R. S., andR. O. Stern: A case of the progressive hypertrophic polyneuritis of Déjérine and Sottas with pathological examination. Brain52, 84–107 (1929).

  9. Dejerine, J., etJ. Sottas: Sur la névrite interstitielle hypertrophique et progressive de l'enfance. C. R. Soc. Biol. (Paris)45, 63–96 (1893).

  10. —, etA. Thomas: Névrite interstitielle et hypertrophique de l'enfance. Nouv. Incon. Salpètr.19, 477–509 (1906).

  11. Dereux, J.: La maladie de Refsum. Rev. neurol.109, 599–608 (1963).

  12. Dinkler: Zur Kasuistik der multiplen Herdsklerose des Gehirns und Rückenmarks. Dtsch. Z. Nervenheilk.26, 233–247 (1904).

  13. Dyck, P. J.: Histologic measurements and fine structure of biopsied sural nerve: normal, and in peroneal muscular atrophy, hypertrophic neuropathy, and congenital sensory neuropathy. Proc. Mayo Clin.41, 742–774 (1966).

  14. Engle, W. K., andG. C. Cunningham: Rapid examination of muscle tissue. Neurology (Minneap.)13, 919–923 (1963).

  15. Evans, D. H. L., andJ. G. Murray: A study of regeneration in a motor nerve with a unimodal fiber diameter distribution. Anat. Rec.126, 311 (1956).

  16. Farber, E., W. H. Sternberg, andC. E. Dunlap: Histochemical localization of specific oxidative enzymes. J. Histochem. Cytochem.4, 254–284 (1956).

  17. Glimstedt, G., andG. Wohlfart: Electron microscopic studies of peripheral nerve regeneration. Lunds. Arsskr. N. F. Avd.56, 16 (1960).

  18. Gombault, A., etMallet: Un cas de tabès. Arch. Méd. exp.1, 385–415 (1889).

  19. Green, L. N., I. Herzog, andD. Aberfeld: A case of hypertrophic neuritis coexisting with dementia and cerebellar degeneration. J. Neuropath. exp. Neurol.24, 682 (1965).

  20. Harris, W., andW. D. Newcomb: A case of relapsing interstitial hypertrophic neuritis. Brain52, 108–116 (1929).

  21. Hess, A.: The fine structure and morphological organization of non-myelinated nerve fibers. Proc. roy. Soc. B144, 496–506 (1956).

  22. Huyley, H. E., andC. Zubay: Preferential staining of nucleic acid containing structures for electron microscopy. J. biophys. biochem. Cytol.11, 273–296 (1961).

  23. Klenk, E., u.W. Kahlke: Über das Vorkommen der 3,7,11,15-Tetramethylhexadecansaure (Phytansäure) in den Cholesterinestern und anderen Lipidfraktionen der Organe bei einem Krankheitsfall unbekannter Genese (Verdacht auf Heredopathia atactica polyneuritiformis — Refsum-Syndrome). Hoppe-Seylers Z. physiol. Chem.333, 133–139 (1963).

  24. Krücke, W.: Die mucoide Degeneration der peripheren Nerven. Virchows Arch. path. Anat.304, 442–463 (1939).

  25. Long, E.: Atrophie musculaire progressive type Aran-Duchenne de nature névritique. Nouv. Incon. Sapètr.25, 281 (1912).

  26. Luft, J. H.: Improvement in epoxy resin embedding methods. J. biochem. biophysic. Cytol.9, 408–414 (1961).

  27. Manheimer, L. H., andA. M. Seligman: Improvement in the method for the histochemical demonstration of alkaline phosphatase and its use in a study of normal and neoplastic tissues. J. nat. Cancer Inst.9, 181–199 (1948).

  28. Marie, M. P.: Forme speciale de névrite interstitielle hypertrophique progressive de l'enfance. Rev. neurol.14, 557–560 (1906).

  29. Marie, P., etI. Bertrand: Contribution à l'anatomie pathologique de la névrite hypertrophique familiale. Ann. Méd.5, 209 (1918).

  30. Masson, P.: Experimental and spontaneous Schwannomas (peripheral gliomas). Amer. J. Path.8, 368–388 (1932).

  31. Nachlas, M. M., andA. M. Seligman: The histochemical demonstration of esterase. J. nat. Cancer Inst.8, 415–425 (1949).

  32. —,K. C. Tsou, E. De Souza, C. S. Cheng, andA. M. Seligman: Cytochemical demonstration of succinic dehydrogenase by the use of a new p-nitrophenyl substituted dietetrazole. J. Histochem. Cytochem.5, 420–425 (1957).

  33. Nageotte, J.: Sheaths of the peripheral nerves: Nerve degeneration and regeneration, vol. I, pp. 189–240. In:Penfield, W.: Cytol. and Cell Path. New York: P. B. Hoeber, Inc. 1932.

  34. Nathaniel, E. J. H., andD. C. Pease: Collagen and basement membrane formation by schwann cells during nerve regeneration. J. Ultrastruct. Res.9, 550–560 (1963).

  35. Reynolds, E. S.: The use of lead citrate in electron microscopy. J. Cell Biol.17, 208 (1963).

  36. Seligman, A. M., K. C. Tsou, R. B. Cohen, andS. H. Rutenberg: Histochemical demonstration of β-D-glucuronidase with a synthetic substrate. J. Histochem. Cytochem.2, 209–229 (1954).

  37. Russell, W. R., andH. G. Garland: Progressive hypertrophic polyneuritis with case reports. Brain53, 376–384 (1930).

  38. Shanthaveerappa, T. R., andG. H. Bourne: Perineural epithelium: A new concept of its role in the integrity of the peripheral nervous system. Science154, 1464–1467 (1966).

  39. Slauck, D. S.: Neuritis interstitialis bzw. Hypertrophica progressiva. Klin. Wschr.8, 927–929 (1929).

  40. Sorgo, J.: Zur Histologie und Klinik der Neurofibrome nebst Bemerkungen über das Verhalten der Patellarreflexe bei Querschnittsläsionen des Rückenmarkes im unteren Brustmark. Virchows Arch. path. Anat.170, 399–428 (1902).

  41. Thomas, P. K.: Changes in the endoneurial sheaths of peripheral myelinated nerve fibers during Wallerian degeneration. J. Anat. (Lond.)98, 175–182 (1964).

  42. Thomas, P. K. andR. G. Lascelles: Schwann cell abnormalities in diabetic neuropathy. Lancet1965 I, 1335–1357.

  43. ——: Hypertrophic neuropathy. Quart. J. Med. (N.S.)36, 223–237 (1967).

  44. Wachstein, M., andE. Meisel: Histochemistry of hepatic phosphatases at physiologic pH. Amer. J. clin. Path.27, 13–23 (1957).

  45. Webster, H. de F., J. M. Schröder, A. K. Asbury, andR. D. Adams: The role of Schwann cells in the formation of “onion bulbs” found in chronic neuropathies. J. Neuropath. exp. Neurol.26, 276–299 (1967).

  46. Weiss, P., M. V. Edds, Jr., andM. Cavanaugh: The effect of terminal connections on the caliber of nerve fibers. Anat. Rec.92, 215–233 (1945).

  47. Weller, R. O.: An electron microscopic study of hypertrophic neuropathy of Déjérine and Sottas. J. Neurol. Neurosurg. Psychiat.30, 111–125 (1967).

  48. Wood, G. C.: The formation of fibrils from collagen solutions III. Effect of chondroitin sulphate and some other naturally occurring polyanions on the rate of formation. Biochem. J.75, 605–612 (1960).

  49. Wolf, A., A. H. Rubinowitz, andS. C. Burchell: Interstitial hypertrophic neuritis of Déjérine and Sottas. Report of three cares. Bull. Neurol. Inst. N.Y.2, 373–428 (1932).

  50. Yokomori, K.: Über Neuritis interstitialis hypertrophica et progressiva (Déjérine u. Sottas) mit einem Sektionsbefund. Mitt. Medizin. Fak. Kaiserl. Univers. Tokyo15, 1–48 (1916).

  51. Zacks, S. I.: Histochemical and ultrastructure observations on “Hyalin degeneration” in severely atrophic muscle. J. Histochem. Cytochem. (Abstr.) (1967).

  52. Zacks, S. I., H. Lipshutz, S. Malkind andF. A. Elliott: Hyaline degeneration in severely atrophic muscle. Histochemical and electron microscopic observations. Brain (in press).

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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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Key-words

  • Hypertrophic Neuritis
  • Onion Bulb Structures
  • Electron Microscopy
  • Segmental Demyelination
  • Schwann Cell Lesion