Diseases

Arnold-Chiari Malformation

A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS, CONGENITAL; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46)

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

A group of congenital malformations involving the brainstem, cerebellum, upper spinal cord, and surrounding bony structures. Type II is the most common, and features compression of the medulla and cerebellar tonsils into the upper cervical spinal canal and an associated MENINGOMYELOCELE. Type I features similar, but less severe malformations and is without an associated meningomyelocele. Type III has the features of type II with an additional herniation of the entire cerebellum through the bony defect involving the foramen magnum, forming an ENCEPHALOCELE. Type IV is a form a cerebellar hypoplasia. Clinical manifestations of types I-III include TORTICOLLIS; opisthotonus; HEADACHE; VERTIGO; VOCAL CORD PARALYSIS; APNEA; NYSTAGMUS, CONGENITAL; swallowing difficulties; and ATAXIA. (From Menkes, Textbook of Child Neurology, 5th ed, p261; Davis, Textbook of Neuropathology, 2nd ed, pp236-46)

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Synonyms

36 entry terms
  • Arnold-Chiari Deformity
  • Arnold-Chiari Syndrome
  • Malformation, Arnold-Chiari
  • Arnold Chiari Deformity
  • Arnold Chiari Malformation
  • Arnold Chiari Syndrome
  • Deformity, Arnold-Chiari
  • Malformation, Arnold Chiari
  • Syndrome, Arnold-Chiari
  • Arnold-Chiari Malformation, Type 1
  • Arnold-Chiari Malformation, Type 2
  • Arnold-Chiari Malformation, Type 3
  • Arnold-Chiari Malformation, Type 4
  • Arnold-Chiari Malformation, Type I
  • Arnold-Chiari Malformation, Type II
  • Arnold-Chiari Malformation, Type III
  • Arnold-Chiari Malformation, Type IV
  • Chiari Malformation Type 2
  • Chiari Malformation Type I
  • Chiari Malformation Type II
  • Type I Arnold-Chiari Malformation
  • Type II Arnold-Chiari Malformation
  • Type III Arnold-Chiari Malformation
  • Type IV Arnold-Chiari Malformation
  • Arnold Chiari Malformation, Type 1
  • Arnold Chiari Malformation, Type 2
  • Arnold Chiari Malformation, Type 3
  • Arnold Chiari Malformation, Type 4
  • Arnold Chiari Malformation, Type I
  • Arnold Chiari Malformation, Type II
  • Arnold Chiari Malformation, Type III
  • Arnold Chiari Malformation, Type IV
  • Type I Arnold Chiari Malformation
  • Type II Arnold Chiari Malformation
  • Type III Arnold Chiari Malformation
  • Type IV Arnold Chiari Malformation

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Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

Indexing Annotation

do not confuse with CHIARI'S SYNDROME see BUDD-CHIARI SYNDROME

MeSH Record

History Note

2000(1963)

MeSH Hierarchy

Tree Numbers

  1. C10w.500w.680w.291w
  2. C16w.131w.666w.680w.291w

MeSH Record

NLM Classification

WL 101

AMA Style

References

  1. National Library of Medicine. Arnold-Chiari Malformation. Medical Subject Headings (MeSH). 2026. Unique ID D001139. http://id.nlm.nih.gov/mesh/2026/D001139
  2. Arnold-Chiari Malformation. In: Wikipedia. https://en.wikipedia.org/wiki/Chiari_malformation
  3. Arnold-Chiari Malformation. In: Wikidata. https://www.wikidata.org/wiki/Q1071707