Skip to main content
Log in

Infantile neuroaxonal dystrophy or Seitelberger's disease

IV. Autonomic nervous system involvement: Electron microscopic study in two siblings

  • Short Original Communications
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Summary

The autonomic nervous system is studied by electron microscopy for the first time in two siblings born of North African consanguineous parents and affected by infantile neuroaxonal dystrophy. The changes already reported in dystrophic axons of the central and peripheral nervous system, are seen in the myenteric plexus of rectum mucosa. The authors stress the diffuse involvement of the nervous tissue in this degenerative disorder of still unknown nature.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  • Berard-Badier, M.: Dystrophie neuroaxonale infantile. I. Ultrastructure des sphéroides. Rapport du 6 me Congr. Internat. de Neuropathol., pp. 101–102. Paris: Masson 1970

    Google Scholar 

  • Berard-Badier, M., Gambarelli, D., Pinsard, N., Hassoun, J., Toga, M.: Infantile neuroaxonal dystrophy or Seitelberger's disease. II. Peripheral nerve involvement: electron microscopic study in one case. Acta neuropath. (Berl.) Suppl. V, 30–39 (1971)

    Google Scholar 

  • Berard-Badier, M., Toga, M.: Infantile neuroaxonal dystrophy (Seitelberger's disease)-autonomic nervous system involvement. IRCS International research Communications System (73-10) 5-11–7 Oct. 1973

  • Berard-Badier, M., Tripier, M. F., Pinsard, N., Bernard, R.: A case of infantile neuroaxonal dystrophy. Strabismus' 69, pp. 82–85. London: Kimpton 1970

    Google Scholar 

  • Cowen, D., Olmstead, E. V.: Infantile neuroaxonal dystrophy. J. Neuropath. exp. Neurol.22, 175–236 (1963)

    Google Scholar 

  • Defendini, R., Markesbery, W. R., Mastri, A. R., Dufy, P. E.: Hallervorden-Spatz disease and infantile neuroaxonal dystrophy. Ultrastructural observations, anatomical pathology and nosology. J. neurol. Sci.20, 7–23 (1973)

    Google Scholar 

  • Duncan, C., Strub, R., McGarry, P., Duncan, D.: Peripheral nerve biopsy as an aid to diagnosis in Infantile neuroaxonal dystrophy. Neurology (Minneap.)20, 1024–1032 (1970)

    Google Scholar 

  • Gilman, S., Barrett, R. E.: Hallervorden-Spatz disease and infantile neuroaxonal dystrophy. J. neurol. Sci.19, 189–205 (1973)

    Google Scholar 

  • Haberland, C., Brunngraber, E. G., Witting, L. A.: Infantile neuroaxonal dystrophy. Neuropathological and biochemical study of a case. Arch. Neurol. (Chic.)26, 391–402 (1972)

    Google Scholar 

  • Jellinger, K.: Neuroaxonal dystrophy: Its natural history and related disorders. Progress in Neuropathology, Vol. II. H. M. Zimmerman, Ed. New York: Grune and Stratton 1973

    Google Scholar 

  • Kamoshita, S., Landing, B. J.: Distribution of lesions in myenteric plexus and gastrointestinal mucosa in lipidoses and other neurologic disorders in children. Amer. J. clin. Path.49, 312–318 (1968)

    Google Scholar 

  • Lampert, P.: A comparative electron microscopy study of reactive, degenerating, regenerating and dystrophic axons. J. Neuropath. exp. Neurol.26, 345–368 (1967)

    Google Scholar 

  • Martin, L., Trelles, L., Martin, J. J.: Evolution clinique de la dystrophie neuro-axonale infantile à la lumière de la chronologie des Atrophies systématisées sous-jacentes. J. neurol. Sci.15, 439–455 (1972)

    Google Scholar 

  • Toga, M., Berard-Badier, M., Gambarelli-Dubois, D.: La dystrophie neuroaxonale infantile ou maladie de Seitelberger. Etude clinique, histologique et ultrastructurale de deux observations. Acta neuropath. (Berl.)15, 327–350 (1970)

    Google Scholar 

  • Toga, M., Berard-Badier, M., Gambarelli, D., Pinsard, N., Hassoun, J.: Un cas de dystrophie neuroaxonale infantile ou maladie de Seitelberger. III. Etude ultrastructurale du muscle strié. Acta neuropath. (Berl.)18, 327–341 (1971a)

    Google Scholar 

  • Toga, M., Berard-Badier, M., Gambarelli, D., Pinsard, N., Hassoun, J.: Le muscle strié dans la maladie de Seitelberger ou Dystrophie neuroaxonale infantile: étude ultrastructurale. In Actualités de Pathologie Neuro-musculaire, pp. 187–190. Paris: Expansion scientifique 1971b

    Google Scholar 

  • Seitelberger, F.: Eine unbekannte Form von infantiler Lipoidspeicher-Krankheit des Gehirns. Proc. 1st. Congr. Neuropath., Vol. 3, pp. 323–333. Turin: Rosenberg et Sellier 1952

    Google Scholar 

  • Seitelberger, F.: Neuropathological conditions related to neuroaxonal dystrophy. Acta neuropath. (Berl.) Suppl.V, 17–29 (1971)

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Berard-Badier, M., Toga, M., Gambarelli, D. et al. Infantile neuroaxonal dystrophy or Seitelberger's disease. Acta Neuropathol 28, 261–267 (1974). https://doi.org/10.1007/BF00719031

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00719031

Key words

Navigation