Arnold-Chiari malformation type II

What is Arnold-Chiari malformation type II?

A rare, central nervous system malformation characterized by caudal displacement of the cerebellum, pons, medulla and fourth ventricle through the foramen magnum into the spinal canal, and is typically associated with myelomeningocele. Variable other central nervous system abnormalities might be present (partial or complete agenesis of the corpus callosum, a small fourth ventricle, obstructive hydrocephalus, falx and tentorium defects, and polygyria). Symptoms include hypotonia, apnea with cyanosis, dysphagia, opisthotonus, nystagmus, spasticity, ataxia, and occipital headache.

Other condition names

  • Arnold-Chiari malformation type 2
  • Chiari malformation type 2
  • Chiari malformation type II

Inheritance type

Not applicable

Age of Onset

  • Childhood
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