Congenital Malformations of the Central Nervous System

  • Gerald S. Golden
Part of the Topics in Pediatrics book series (TIPE)


Malformations of the central nervous system (CNS) are among the most common of the major congenital anomalies and are an important source of chronic disability in childhood. Exact incidence figures are difficult to determine, as they vary with geographical location, the population group under study, and the epidemiological techniques used. In addition, there are unexplained changes in incidence over long periods of time. It now appears, for instance, that the frequency of occurrence of spina bifida has been decreasing.1


Mental Retardation Down Syndrome Congenital Malformation Spina Bifida Fourth Ventricle 
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  1. 1.
    Windham, G. C., and Edmonds, L. D., 1982, Current trends in the incidence of neural tube defects, Pediatrics 70: 333–337.PubMedGoogle Scholar
  2. 2.
    Kurtzke, J. F., and Goldberg, I. D., 1973, The distribution of deaths from congenital malformations of the nervous system, Neurology 23: 483–496.PubMedGoogle Scholar
  3. 3.
    Bell, J. E., 1979, Central nervous system defects in early human abortuses, Dev. Med. Child. Neurol. 21: 321–332.PubMedCrossRefGoogle Scholar
  4. 4.
    Zwilling, E., 1955, Teratogenesis, in: Analysis of Development ( B. H. Willier, P. A. Weiss, and V. Hamburger, eds.), WB Saunders, Philadelphia, pp. 699–719.Google Scholar
  5. 5.
    Wilson, J. G., 1973, Mechanisms of teratogenesis, Am. J. Anat. 136: 129–131.PubMedCrossRefGoogle Scholar
  6. 6.
    Smithells, R. W., 1982, Neural tube defects: Prevention by vitamin supplements, Pediatrics 69: 498–499.PubMedGoogle Scholar
  7. 7.
    Bertoshesky, L. E., Haller, J., Scott, R. M., and Wojick, C., 1985, Seizures in children with meningomyelocele, Am. J. Dis. Child. 139: 400–402.Google Scholar
  8. 8.
    Charney, E. B., Weller, S. C., Sutton, L. N., Bruce, D. A., and Schut, L. B., 1985, Management of the newborn with myelomeningocele: Time for a decision-making process, Pediatrics 75: 58–64.PubMedGoogle Scholar
  9. 9.
    McI,one, D.G., Czyzewski, D., Raimondi, A. J., and Sommers, R. C., 1982, Central nervous system infections as a limiting factor in the intelligence of children with myelomeningocele, Pediatrics 70: 338–342.Google Scholar
  10. 10.
    Ehrlich, O., and Brem, A. S., 1982, A prospective comparison of urinary tract infections in patients treated with either clean intermittent catheterization or urinary diversion, Pediatrics 70: 665–669.PubMedGoogle Scholar
  11. 11.
    Uehling, D. T., Smith, J., Meyer, J., and Bruskewitz, R., 1985, Impact of an intermittent catheterization program on children with myelomeningocele, Pediatrics 76: 892–895.PubMedGoogle Scholar
  12. 12.
    Bjerkedal, T., Czeizel, A., Goujard, J., Kallen, B., Mastroiacova, P., Nevin, N., Oakley, G., and Robert, E., 1982, Valproic acid and spina bifida, Lancet 2: 1096.PubMedCrossRefGoogle Scholar
  13. 13.
    Smithells, R. W., 1982, Neural tube defects: Prevention by vitamin supplements, Pediatrics 69: 498–499.PubMedGoogle Scholar
  14. 14.
    Fisher, N. I., and Smith, D. W., 1981, Occipital encephalocele and early gestational hyperthermia, Pediatrics 68: 480–483.PubMedGoogle Scholar
  15. 15.
    Lemire, R. J., Loeser, J. D., Leach, R. W., and Alvord, E. C., 1975, Normal and Abnormal Development of the Human Nervous System, Harper and Row, Hagerstown, MD.Google Scholar
  16. 16.
    Lammer, E. J., Chen, D. T., Hoar, R. M., Agnish, N. D., Benke, P. J., Braun, J. T., Curry, C. J., Fernhoff, P. M., Grix, A. W., Lott, I. T., Richard, J. M., and Sun, S. C., 1985, Retinoic acid embryopathy, N. Engl. J. Med. 313: 837–841.PubMedCrossRefGoogle Scholar
  17. 17.
    Shurtleff, D. B., Foltz, E., and Loeser, J. D., 1973, Hydrocephalus: A definition of its progression and relationship to intellectual function, diagnosis, and complications, Am. J. Dis. Child. 125: 688–693.PubMedGoogle Scholar
  18. 18.
    Martin, H. P., 1970, Microcephaly and mental retardation, Am. J. Dis. Child. 119: 128–131.PubMedGoogle Scholar
  19. 19.
    Wood, J. W., Johnson, K. G., and Omori, Y., 1967, In utero exposure to the Hiroshima atomic bomb, Pediatrics 39: 385–392.PubMedGoogle Scholar
  20. 20.
    Yamazaki, J. N., 1966, A review of the literature on the radiation dosage required to cause manifest central nervous system disturbances from in utero and postnatal exposure, Pediatrics 37 (Suppl.): 877–903.PubMedGoogle Scholar
  21. 21.
    Lemieux, B. G., 1982, Chromosomal aberrations, in: The Practice of Pediatric Neurology ( K. S. Swaiman and F. S. Wright, eds.), C.V. Mosby, St. Louis, pp. 344–402.Google Scholar
  22. 22.
    de la Cruz, F., 1985, Fragile X syndrome, Am. J. Ment. Def. 90: 119–123.Google Scholar
  23. 23.
    Shuper, A., Merlob, P., Grunebaum, M., and Reisner, S. H., 1985, The incidence of isolated craniosynostosis in the newborn infant, Am. J. Dis. Child. 139: 85–86.PubMedGoogle Scholar
  24. 24.
    Noetzel, M.J., Marsh, J. L., Palkes, H., and Gado, M., 1985, Hydrocephalus and mental retardation in craniosynostosis, J. Pediatr. 107: 885–892.PubMedCrossRefGoogle Scholar
  25. 25.
    Shepard, T. H., 1982, Detection of human teratogenic agents, J. Pediatr. 101: 810–815.PubMedCrossRefGoogle Scholar

Additional Reading

  1. Gorlin, R. J., Pindborg, J. J., and Cohen, M. M., 1976, Syndromes of the Head and Neck, 2nd ed., McGraw-Hill, New York.Google Scholar

Copyright information

© Plenum Publishing Corporation 1987

Authors and Affiliations

  • Gerald S. Golden
    • 1
  1. 1.Department of Neurology, The Health Science CenterUniversity of Tennessee, MemphisMemphisUSA

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