Preferred Name

Arnold-Chiari Malformation [Disease/Finding]
Synonyms

Arnold-Chiari Malformation

Arnold-Chiari Syndrome

Arnold-Chiari Deformity

Malformation, Arnold-Chiari

ID

http://purl.bioontology.org/ontology/NDFRT/N0000000467

altLabel

Arnold-Chiari Malformation

Arnold-Chiari Syndrome

Arnold-Chiari Deformity

Malformation, Arnold-Chiari

cui

C0003803

MESH DEFINITION

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)

MESH DUI

D001139

MeSH name

Arnold-Chiari Malformation

MESH UI

M0001704

NDFRT kind

DISEASE_KIND

notation

N0000000467

NUI

N0000000467

prefLabel

Arnold-Chiari Malformation [Disease/Finding]

SNOMED CID

253184003

tui

T019

subClassOf

http://purl.bioontology.org/ontology/NDFRT/N0000002161

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