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)

Annotation

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

nepleť s Chiariho syndromem, ten viz BUDDŮV-CHIARIHO SYNDROM

DUI

D001139 MeSH Browser

CUI

M0001704

History note

2000(1963)

Public note

2000; see ARNOLD-CHIARI DEFORMITY 1963-1999; for ARNOLD-CHIARI MALFORMATION see ARNOLD-CHIARI DEFORMITY 1963-1999