Forms of Hindbrain Crowding, Including the Arnold-Chiari Malformation

  • Reinhard L. Friede


The deformities of brain stem and cerebellum known as Arnold-Chiari malformation were first described in nine infants by Cleland (1883). He reported and illustrated the herniation of the vermis and the deformities of medulla oblongata and the tectal plate. Chiari (1891, 1896) described these lesions in detail, also including the ascending nerve roots, and made reference to Cleland’s article. Excerpts of Chiari’s (1891) description were republished (Wilkins and Brody 1971). Chiari’s main concern was classifying cerebellar defomities in hydrocephalic infants. He distinguished three types: type I, herniation of the cerebellar tonsils (see “Chronic Tonsillar Herniations” later in this chapter). Type II is now widely recognized as the Arnold-Chiari malformation. Type III was cervical spina bifida with cerebellar encephalocele. In a second report (1896), on 24 cases, Chiari included cerebellar hypoplasia as type IV. Arnold’s (1894) contribution consists of an infant with myelomeningocele, absence of sacrum, sacral teratoma, and deformed lower extremities. A “ribbon-like” cerebellar herniation extended to the mid-cervical region. Its origin from vermis or tonsils was not specified, nor was there a record of a brain stem deformity. Arnold’s coworkers, Schwalbe and Gredig (1907), proposed the term Arnold-Chiari malformation and suggested the name “Arnold’s malformation” for the cerebellar hernia and “Chiari’s malformation” for the brain stem deformity. Their paper had considerable impact even though the designations were clearly wrong and in contradiction to the information available to the authors.


Posterior Fossa Spina Bifida Chiari Malformation Cerebellar Tonsil Tonsillar Herniation 
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Copyright information

© Springer-Verlag Berlin Heidelberg 1989

Authors and Affiliations

  • Reinhard L. Friede
    • 1
  1. 1.Zentrum Neurologische Medizin, Abteilung NeuropathologieGeorg-August-Universität GöttingenGöttingenGermany

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