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Genetic and Rare Diseases Information Center (GARD)

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Chiari malformation type 1


* Not a rare disease
Other Names for this Disease
  • Arnold Chiari malformation type I
  • Chiari malformation type I
  • Chiari type I malformation
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How might Chiari malformation type 1 be treated?

Some individuals with Chiari malformation type 1 are asymptomatic and do not require treatment.[1] Individuals who have minimal symptoms, without syringomyelia, can typically be treated conservatively. Mild neck pain and headaches can usually be treated with pain medications, muscle relaxants, and the occasional use of a soft collar.[2]

Individuals with more severe symptoms may be in need of surgery.[2] Surgery is the only treatment available to correct functional disturbances or halt the progression of damage to the central nervous system.[1] The goals of surgical treatment are decompression of the point where the skull meets the spine (the cervicomedullary junction) and restoration of normal flow of cerebrospinal fluid in the region of the foramen magnum (the hole in the bottom of the skull where the spinal cord passes to connect to the brain).[2] Prognosis after surgery for the condition is generally good and typically depends on the extent of neurological deficits that were present before the surgery. Most individuals have a reduction of symptoms and/or prolonged periods of relative stability. More than one surgery may be needed to treat the condition.[1][2]
Last updated: 3/12/2012

  1. Chiari Malformation Fact Sheet. NINDS. February 1, 2012; Accessed 3/11/2012.
  2. Peyman Pakzaban. Chiari Malformation. eMedicine. March 2, 2012; Accessed 3/11/2012.

Clinical Trials & Research for this Disease

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.