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NINDS Chiari Malformation Information Page

Synonym(s):   Arnold-Chiari Malformation
Condensed from Chiari Malformation Fact Sheet

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What is Chiari Malformation?

Chiari malformations (CMs) are structural defects in the cerebellum, the part of the brain that controls balance. When the indented bony space at the lower rear of the skull is smaller than normal, the cerebellum and brainstem can be pushed downward. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid (the liquid that surrounds and protects the brain and spinal cord) and can cause a range of symptoms including dizziness, muscle weakness, numbness, vision problems, headache, and problems with balance and coordination. There are three primary types of CM. The most common is Type I, which may not cause symptoms and is often found by accident during an examination for another condition. Type II (also called Arnold-Chiari malformation) is usually accompanied by a myelomeningocele-a form of spina bifida that occurs when the spinal canal and backbone do not close before birth, causing the spinal cord to protrude through an opening in the back. This can cause partial or complete paralysis below the spinal opening. Type III is the most serious form of CM, and causes severe neurological defects. Other conditions sometimes associated with CM include hydrocephalus, syringomyelia, and spinal curvature.

Is there any treatment?

Medications may ease certain symptoms, such as pain. Surgery is the only treatment available to correct functional disturbances or halt the progression of damage to the central nervous system. More than one surgery may be needed to treat the condition.

What is the prognosis?

Many people with Type I CM are asymptomatic and do not know they have the condition. Many patients with the more severe types of CM and have surgery see a reduction in their symptoms and/or prolonged periods of relative stability, although paralysis is generally permanent.

What research is being done?

The NINDS supports research on disorders of the brain and nervous system such as Chiari malformations. The goals of this research are to increase scientific understanding of these disorders and to find ways to prevent, treat, and, ultimately, cure them.

NIH Patient Recruitment for Chiari Malformation Clinical Trials

Organizations

Column1 Column2
March of Dimes
1275 Mamaroneck Avenue
White Plains, NY   10605
askus@marchofdimes.com
http://www.marchofdimes.com
Tel: 914-997-4488 888-MODIMES (663-4637)
Fax: 914-428-8203

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT   06810
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

Spina Bifida Association
4590 MacArthur Blvd. NW
Suite 250
Washington, DC   20007-4266
sbaa@sbaa.org
http://www.spinabifidaassociation.org
Tel: 202-944-3285 800-621-3141
Fax: 202-944-3295

American Syringomyelia & Chiari Alliance Project (ASAP)
P.O. Box 1586
Longview, TX   75606-1586
info@asap.org
http://www.asap.org
Tel: 903-236-7079 800-ASAP-282 (272-7282)
Fax: 903-757-7456

Chiari & Syringomyelia Foundation
29 Crest Loop
Staten Island, NY   10312
info@CSFinfo.org
http://www.csfinfo.org
Tel: 718-966-2593
Fax: 718-966-2593 (Call First)

 
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Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



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Last updated February 1, 2012