Skip to main content

Possible evidence for secondary degeneration of central nervous system in the pathogenesis of anencephaly and brain dysraphia

A study in young human fetuses

Virchows Archiv A volume 384, pages 285–294 (1979)Cite this article

Summary

In an attempt to help elucidate pathogenetically those human cases exemplifying secondary degeneration of the neural tube causing brain dysraphia, macroscopic and histologic observations of two young human fetuses are described. A nine-week-old anencephalic fetus exhibited an absence of spinal cord (amyelia) with retention of neural crest derivatives (dorsal root ganglion cells and their processes, and sympathetic ganglia) implying the presence of a neural tube in early gestation. The second, ten-week-old exencephalic case exhibited restricted brain hemorrhage and necrosis of the telencephalon and brain stem amongst otherwise normal brain and spinal cord tissue. These two young fetal cases may represent examples of a previously normal neural tube which has undergone degeneration at a stage where neural crest has already undergone differentiation, and thus distinguishes them from cases of complete dysraphism which probably results from primary degeneration during neurulation.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

43,34 €

Price includes VAT (Finland)
Tax calculation will be finalised during checkout.

References

  • Brent, R.L.: Radiations and other physical agents. In: Handbook of teratology (J.G. Wilson and F.C. Fraser, eds.), vol. 1, pp. 153–223. New York: Plenum Press 1977

    Google Scholar 

  • Carter, C.O.: Clues to the aetiology of neural tube malformations. Dev. Med. Child Neurol. 16 (6 Suppl. 32), 3–15 (1974)

    Google Scholar 

  • Chaurasia, B.D.: Forebrain in human anencephaly. Anat. Anz. 142, 471–478 (1977)

    Google Scholar 

  • Dekaban, A.S.: Anencephaly in early human embryos. J. Neuropath. Exp. Neurol. 22, 533–548 (1963)

    Google Scholar 

  • Dekaban, A.S.: Abnormalities in children exposed to X-irradiation during various stages of gestation: tentative timetable of radiation injury to the human fetus, Part 1. J. Nucl. Med. 9, 471–477 (1968)

    Google Scholar 

  • Elwood, J.H.: Anencephaly and spina bifida in the British Isles. In: Birth defects. Risks and consequences (S. Kelly, E.B. Hook, D.T. Janerich and I.H. Porter, eds.), pp. 21–39. New York: Academic Press 1976

    Google Scholar 

  • Elwood, J.M.: Anencephalus and spina bifida in North America. In: Birth defects. Risks and consequences (S. Kelly, E.B. Hook, D.T. Janerich and I.H. Porter, eds.), pp. 3–19. New York: Academic Press 1976

    Google Scholar 

  • Gardner, W.J.: Rupture of the neural tube. Arch. Neurol. 4, 1–7 (1961)

    Google Scholar 

  • Gardner, W.J.: The dysraphic states: from syringomyelia to anencephaly. Amsterdam: Excerpta Medica 1973

    Google Scholar 

  • Hanaway, J., Welch, G.: Anencephaly: a review and interpretation in terms of modern experimental embryology. Dis. Nerv. Syst. 31, 527–533 (1970)

    Google Scholar 

  • Harris, R.E.: Viral teratogenesis: a review with experimental and clinical perspectives. Am. J. Obstet. Gynecol. 119, 996–1008 (1974)

    Google Scholar 

  • Hicks, S.P., D'Amato, C.J., Lowe, M.J.: The development of the mammalian nervous system. I. Malformations of the brain especially the cerebral cortex, induced in rats by radiation. II. Some mechanisms of the malformations of the cortex. J. Comp. Neurol. 113, 435–469 (1959)

    Google Scholar 

  • Langman, J., Welch, G.W.: Effect of Vitamin A on development of the central nervous system. J. Comp. Neurol. 128, 1–16 (1966)

    Google Scholar 

  • Lemire, R.J., Loeser, J.D., Leech, R.W., Alford, Jr., E.C.: Normal and abnormal development of the human nervous system. New York: Harper and Row 1975

    Google Scholar 

  • Mall, F.P.: Determination of the age of human embryos and fetuses. In: Manual of human embryology (F. Keibel and F.P. Mall, eds.), vol. 1, pp. 180–201. Philadelphia: Lippincott 1910

    Google Scholar 

  • Mann, Ida: Developmental abnormalities of the eye, pp. 50–55. London: British Medical Assn. 1957

    Google Scholar 

  • Marin-Padilla, M.: Morphogenesis of anencephaly and related malformations. In: Current topics in pathology, vol. 51, pp. 145–174. Berlin: Springer 1970

    Google Scholar 

  • Menashi, M., Ornoy, A., Cohen, M.M.: Anencephaly in trisomy 18: related or unrelated? Teratology 15, 325–328 (1977)

    Google Scholar 

  • Naggan, L.: Anencephaly and spina bifida in Israel. Pediatrics 47, 577–586 (1971)

    Google Scholar 

  • O'Rahilly, R., Gardner, E.: The timing and sequence of events in the development of the human nervous system during the embryonic period proper. Z. Anat. EntwGesch 134, 1–12 (1971)

    Google Scholar 

  • Ornoy, A., Segal, S., Nishmi, M., Simcha, A., Polishuk, W.Z.: Fetal and placental pathology in gestational rubella. Am. J. Obstet. Gynecol. 116, 949–956 (1973)

    Google Scholar 

  • Patten, B.M.: Embryological stages in the establishing of myeloschisis with spina bifida. Amer. J. Anat. 93, 365–395 (1953)

    Google Scholar 

  • Patten, B.M.: Human embryology. New York: McGraw-Hill 1968

    Google Scholar 

  • Rugh, R., Grupp, E.: Exencephalia following X-irradiation of the pre-implantation mammalian embryo. J. Neuropath. Exp. Neurol. 18, 468–481 (1959)

    Google Scholar 

  • Sabato, S.: Anencephaly, a congenital malformation of the central nervous system, as demonstrated by two infants born at the Hadassah Medical Center in 1971. M.D. Thesis, unpublished (1973)

  • de Vries, E.: Description of a young anencephalic and amyelic embryo. Anat. Rec. 36, 293–317 (1927)

    Google Scholar 

  • Warkany, J.: Congenital malformations. Chicago: Year Book Medical Publishers, Inc. 1971

    Google Scholar 

  • Warren, H.S.: Acrania induced by anencephaly. Anat. Rec. 111, 653–667 (1951)

    Google Scholar 

  • Washburn, W.W.: Wound healing as a problem of growth. In: Fundamental aspects of normal and malignant growth (W.W. Nowinski, ed.), pp. 664–711. New York: Elsevier Publ. 1960

    Google Scholar 

  • Willis, R.A.: The borderland of embryology and pathology, pp. 147–163. London: Butterworths 1962

    Google Scholar 

Download references

Author information

Affiliations

  1. Department of Anatomy and Embryology, Hebrew University-Hadassah Medical School, P.O.Box 1172, Jerusalem, Israel

    Donald Ganchrow & Asher Ornoy

Authors
  1. Donald Ganchrow
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Asher Ornoy
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Supported in part by Myra Kurland Heart Fund Heart Research

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Ganchrow, D., Ornoy, A. Possible evidence for secondary degeneration of central nervous system in the pathogenesis of anencephaly and brain dysraphia. Virchows Arch. A Path. Anat. and Histol. 384, 285–294 (1979). https://doi.org/10.1007/BF00428230

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00428230

Key words

  • Anencephalus
  • Amyelia
  • Secondary degeneration
  • Central nervous system: abnormalities