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)

Also Known As:

Arnold-Chiari Syndrome; Syndrome, Arnold-Chiari; Arnold-Chiari Deformity; 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; Malformation, Arnold-Chiari; Type I Arnold-Chiari Malformation; Type II Arnold-Chiari Malformation; Type III Arnold-Chiari Malformation; Type IV Arnold-Chiari Malformation; Arnold Chiari Deformity; 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; Arnold Chiari Syndrome; Deformity, Arnold-Chiari; Malformation, Arnold Chiari; Type I Arnold Chiari Malformation; Type II Arnold Chiari Malformation; Type III Arnold Chiari Malformation; Type IV Arnold Chiari Malformation

Nervous System Diseases: 14178
- Nervous System Malformations: 35
  - Neural Tube Defects: 4299
    - Arnold-Chiari Malformation: 309
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Congenital Abnormalities: 25723
  - Nervous System Malformations: 35
    - Neural Tube Defects: 4299
      - Arnold-Chiari Malformation: 309

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