The Arnold-Chiari Malformation (Cleland-Chiari Malformation)

  • Reinhard L. Friede


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.


Brain Stem Posterior Fossa Spina Bifida Medulla Oblongata Chiari Malformation 
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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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