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The Arnold-Chiari Malformation (Cleland-Chiari Malformation)

  • Reinhard L. Friede

Abstract

The deformities of brain stem and cerebellum generally known as ArnoldChiari malformation were first described by Cleland (1883) for one infant of a series of 9 presenting with various combinations of spina bifida cystica, encephalocele and anencephaly. Cleland reports and illustrates the herniation of the vermis and the deformities of the medulla oblongata and tectal plate. Chiari (1891, 1896) described these lesions in considerable detail, including the ascending direction of nerve roots, and made reference to Cleland’s article; excerpts of Chiari’s (1891) original description were recently translated and republished (Wilkins and Brody, 1971). Chiari was concerned mainly with the types of cerebellar deformities, of which he distinguished three types: The first, consisting of herniation of the cerebellar tonsils is considered more closely under “Chronic Tonsillar Herniation” later in this chapter; type II is now widely recognized as the Arnold-Chiari malformation; type III was an instance of cervical spina bifida with cerebellar encephalocele. Twentyfour cases form the basis of the second report (1896), in which a fourth type, cerebellar hypoplasia, was added. Arnold’s (1894) contribution consists of a report on an infant with myelomeningocele and absence of sacrum, sacral teratoma, and deformities of the lower extremities.

Keywords

Brain Stem Posterior Fossa Spina Bifida Medulla Oblongata Chiari Malformation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. Adams, R. D., Schatzki, R., Scoville, W. B.: Arnold-Chiari malformation: diagnosis, demonstration by intraspinal lipiodol and successful surgical treatment. New Engl. J. Med. 225: 125–131, 1941.Google Scholar
  2. Alvord, E. C., Jr.: Tie pathology of hydrocephalus. In: Disorders of the Developing Nervous System, Chapter 14, pp. 343–412. Springfield, Ill.: Ch. C Thomas 1961.Google Scholar
  3. Arnold, J.: Myelocyste, Transposition von Gewebskeimen und Sympodie. Beitr. path. Anat. 16: 1–28, 1894.Google Scholar
  4. Banerji, N. K., Millar, J. H. D.: Chiari malformation presenting in adult life—Its relationship to syringomyelia. Brain 97: 157–168, 1974.PubMedCrossRefGoogle Scholar
  5. Barry, A., Patten, B. M., Stewart, B. H.: Possible factors in the development of the Arnold-Chiari malformation. J. Neurosurg. 14: 285–301, 1957.PubMedCrossRefGoogle Scholar
  6. Blaauw, G.: Defect in posterior arch of atlas in myelomeningocele. Develop. Med. Child. Neurol., Suppl. 25: 113–115, 1971.Google Scholar
  7. Brocklehurst, G.: A quantitative study of a spina bifida foetus. J. Path. 99: 205–211, 1969.PubMedCrossRefGoogle Scholar
  8. Brocklehurst, G.: The pathogenesis of spina bifida: A study of the relationship between observation, hypothesis and surgical incentive. Develop. Med. Child. Neurol. 13: 147–163, 1971.PubMedCrossRefGoogle Scholar
  9. Cameron, A. H.: Arnold-Chiari malformation and other neuroanatomical malformations associated with spina bifida. J. Path. Bact. 73: 195–210, 1957.CrossRefGoogle Scholar
  10. Chiari, H.: Über Veränderungen des Kleinhirns, der Pons und der Medulla oblongata in Folge von congenitaler Hydrocephalie des Großhirns. Dtsch. med. Wschr. 27: 1172–1175, 1891.Google Scholar
  11. Chiari, H.: Über Veränderungen des Kleinhirns, Pons und der Medulla oblongata in Folge von congenitaler Hydrocephalie des Großhirns. Denkschr. Kaiser. Akad. Wiss. Math. Naturw. Classe 63: 71–115, 1896.Google Scholar
  12. Cleland: Contributions of the study of spina bifida, encephalocele and anencephalus.J. Anat. Physiol. 17: 257–292, 1883.PubMedGoogle Scholar
  13. Collier, J.: The false localizing signs of intracranial tumor. Brain 27: 490–508, 1904.CrossRefGoogle Scholar
  14. Cushing, H.: Some experimental and clinical observations concerning states of increased intracranial tension. Amer. J. med. Sci. 124: 375–400, 1902.CrossRefGoogle Scholar
  15. Daniel, P. M., Strich, S. J.: Some observations on the congenital deformity of the central nervous system known as the Arnold-Chiari malformation. J. Neuropath. exp. Neurol. 17: 255–266, 1958.PubMedCrossRefGoogle Scholar
  16. Doran, P. A., Guthkelch, A. N.: Studies in spina bifida cystica. I. General survey and reassessment of the problem. J. Neurol. Neurosurg. Psychiat. 24: 331–345, 1961.Google Scholar
  17. Emery, J. L.: Effects of continual decompression using Holter valve on weights of cerebral hemispheres in children with hydrocephalus and spina bifida cystica. Arch. Dis. Child. 39: 379–383, 1964.PubMedCrossRefGoogle Scholar
  18. Emery, J. L.: Intracranial effects of long-standing decompression of the brain in children with hydrocephalus and meningomyelocele. Develop. Med. Child. Neurol.7: 302–309, 1965.PubMedCrossRefGoogle Scholar
  19. Emery, J. L.: Deformity of the aqueduct of Sylvius in children with hydrocephalus and myelomeningocele. Develop. Med. Child Neurol. Suppl. 32: 40–48, 1974.Google Scholar
  20. Essick, C. R.: The development of the nuclei pontis and the nucleus arcuatus in man. Amer.T. Anat. 13: 25–54, 1912.Google Scholar
  21. Gardner, J. W., Goodall, R. J.: The surgical treatment of Arnold-Chiari malformation in adults. T.Neurosurg. 7: 199–206, 1950.CrossRefGoogle Scholar
  22. Geipel, P.: Zur Kenntnis der Spina bifida des Atlas. Fortschr. Röntgenstr. 42: 583–589, 1930.Google Scholar
  23. Goldstein, F., Kepes, J. J.: The relationship of the Arnold-Chiari malformation to lumbar meningomyeloceles. An experimental study. Vth Internat. Congr. Neuropath.,Excerpta Medica Foundation, Amsterdam, 1965, pp. 734–737.Google Scholar
  24. Gunberg, D. L.: Spina bifida and the Arnold-Chiari malformation in the progeny of trypan blue injected rats. Anat. Rec. 126: 343–367, 1956.PubMedCrossRefGoogle Scholar
  25. Gustafson, W. A., Oldenberg, E.: Neurological significance of platybasia. Arch. Neurol. Psychiat. 44: 1184–1198, 1940.Google Scholar
  26. Ingraham, F. D., Scott, H. W.: Spina bifida and cranium bifidum; Arnold-Chiari malformation; a study of 20 cases. New Engl. J. Med. 229: 108–114, 1943.Google Scholar
  27. Jacobs, E. B., Landing, B. H., Thomas, W., Jr.: Vernicomyelia. Its bearing on theories of genesis of the Arnold Chiari complex. Am. J. Path. 39: 345–353, 1961.PubMedGoogle Scholar
  28. Kruyff, E., Jeffs, R.: Skull abnormalities associated with Arnold-Chiari malformation. Acta radiol. (Diagn.) (Stockh.) 5: 9–24, 1966.Google Scholar
  29. Laurence, K. M.: Some applications of the urinary phenolsulphonphthalein excretion test in hydrocephalus and related conditions. Brain 82: 551–565, 1959.PubMedCrossRefGoogle Scholar
  30. Laurence, K. M.: The natural history of spina bifida cystica. Proc. roy. Soc. Med. 53: 1055–1056, 1960.Google Scholar
  31. List, C. F.: Neurologic syndromes accompanying developmental anomalies of occipital bone, atlas and axis. Arch. Neurol. Psychiat. 45: 577–616, 1941.Google Scholar
  32. Lorber, J.: Systematic ventriculographic studies in infants born with meningomyelocele and encephalocele (The incidence and development of hydrocephalus). Arch. Dis. Child. 36: 381–389, 1961.PubMedCrossRefGoogle Scholar
  33. MacFarlane, A., Maloney, A. F. J.: The appearance of the aqueduct and itsrelationship to hydrocephalus in the Arnold-Chiari malformation. Brain 80: 479–491, 1957.PubMedCrossRefGoogle Scholar
  34. MacKenzie, N. G., Emery, J. L.: Deformities of the cervical cord in children with neurospinal dvsraphism. Develop. Med. Child. Neurol. 13, Suppl. 25: 58–67, 1971.Google Scholar
  35. McConnell, A. A., Parker, H. L.: Deformity of hind-brain associated with internal hydrocephalus; its relation to Arnold-Chiari malformation. Brain 61: 415–429, 1938.CrossRefGoogle Scholar
  36. Margolis, G., Kilham, L. K.: Experimental virus-induced hydrocephalus. J. Neurosurg. 31: 1–9, 1969.PubMedCrossRefGoogle Scholar
  37. Meyer, A.: Herniation of the brain. Arch. Neurol. Psychiat. 4: 387–400, 1920.Google Scholar
  38. Nathan, H., Feuerstein, M.: Angulated course of spinal nerve roots. J. Neurosurg. 32: 349–352, 1970.PubMedCrossRefGoogle Scholar
  39. Ogryzlo, M. A.: Arnold-Chiari malformation. Arch. Neurol. Psvchiat. 48: 30–46. 1942.Google Scholar
  40. Peach, B.: Arnold-Chiari malformation with normal spine. Arch. Neurol. 10: 497–501, 1964.PubMedCrossRefGoogle Scholar
  41. Peach, B.: Arnold-Chiari malformation. Arch. Neurol. 12: 527–535, 1965.PubMedCrossRefGoogle Scholar
  42. Kay, B. S.: Platybasia with involvement of the central nervous system. Ann. Surg. 116: 231–250, 1942.CrossRefGoogle Scholar
  43. Reid, J. D.: Ascending nerve roots. J. Neurol. Neurosurg. Psychiat. 23: 148–155, 1960.PubMedCrossRefGoogle Scholar
  44. Kussell, D. S.: Observations on the pathology of hydrocephalus. London: Her Majesty’s Stationery Office 1968.Google Scholar
  45. Kussell, D. S., Donald, C.: The mechanism of internal hydrocephalus in spina bifida. Brain 58: 203–215, 1935.CrossRefGoogle Scholar
  46. Sandbank, U.: Le syndrome d’Arnold-Chiari. Rev. Neurol. 93: 529–563, 1955.PubMedGoogle Scholar
  47. Schwalbe, E., Gredig, M.: Uber Entwicklungsstörungen des Kleinhirns, Hirnstamms und Halsmarks bei Spina bifida. Beitr. path. Anat. Ziegler 40: 132–194, 1907.Google Scholar
  48. Sieben, R. L., Hamida, M. B., Shulman, K.: Multiple cranial nerve deficits associated with the Arnold-Chiari malformation. Neurology 21: 673–681, 1971.PubMedCrossRefGoogle Scholar
  49. Smith, E. D.: Spina Bifida and the Total Care of Spinal Myelomeningocele. Springfield, Ill.: Ch. C Thomas 1965.Google Scholar
  50. Spillane, J. D., Pallis, C., Jones, A. M.: Developmental abnormalities in the region of the foramen magnum. Brain 80: 11–48, 1957.PubMedCrossRefGoogle Scholar
  51. Variend, S., Emery, J. L.: The weight of the cerebellum in children with myelomeningocele. Develop. Med. Child. Neurol. 15, Suppl. 29: 77–83, 1973.Google Scholar
  52. Vogt, E. C., Wyatt, G. M.: Craniolacunia (Lückenschädel). A report of 54 cases. Radiology 36: 147–153, 1941.Google Scholar
  53. Wilkins, R. H., Brody, I. A.: The Arnold-Chiari malformation. Arch. Neurol. 25: 376–379, 1971.PubMedCrossRefGoogle Scholar
  54. Yu, H. C., Deck, M. D. F.: The clivus deformity of the Arnold-Chiari malformation. Radiology 101: 613–616, 1971.PubMedGoogle Scholar

Copyright information

© Springer-Verlag Wien 1975

Authors and Affiliations

  • Reinhard L. Friede
    • 1
    • 2
  1. 1.Case Western Reserve UniversityClevelandUSA
  2. 2.University of ZurichSwitzerland

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