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Diseases & Conditions

Chiari Malformation

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.

Organizations

March of Dimes

1275 Mamaroneck Avenue
White Plains, NY 10605
Phone: 914.997.4488
Toll-free: 888.MODIMES (663.4637)
Fax: 914.428.8203
Email: askus@marchofdimes.com
Website: www.marchofdimes.com

National Organization for Rare Disorders (NORD)

55 Kenosia Avenue
Danbury, CT 06810
Phone: 203.744.0100
Voice Mail: 800.999.NORD (6673)
Fax: 203.798.2291
Email: orphan@rarediseases.org
Website: www.rarediseases.org

Spina Bifida Association of America

4590 MacArthur Blvd. NW, Suite 250
Washington, DC 20007-4266
Phone: 202.944.3285
Toll-free: 800.621.3141
Fax: 202.944.3295
Email: sbaa@sbaa.org
Website: www.spinabifidaassociation.org

American Syringomyelia Alliance Project (ASAP)

P.O. Box 1586
Longview, TX 75606-1586
Phone: 903.236.7079
Toll-free: 800.ASAP.282 (272.7282)
Fax: 903.757.7456
Email: info@asap.org
Website: www.asap.org

Chiari & Syringomyelia Foundation

29 Crest Loop
Staten Island, NY 10312
Phone: 718.966.2593
Fax: 718.966.2593 (Call First)
Email: info@CSFinfo.org
Website: www.csfinfo.org

Source: National Institutes of Health; National Institute of Neurological Disorders and Stroke

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 2/1/2012...#6008