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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 brain stem 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. Symptoms may change for some individuals depending on buildup of CNS and any resulting pressure on tissue and nerves. CMs are classified by the severity of the disorder and the parts of the brain that protrude into the spinal canal. 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 (a fluid-filled cyst in the spinal cord), 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. Some CMs have no noticeable symptoms and do not interfere with the person's activities of daily living.

What is the prognosis?

Many people with Type I CM are asymptomatic and do not know they have the condition. Many individuals 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.  Current NINDS-funded research includes studies to better understand the genetic factors responsible for the malformation, and  factors that influence the development, progression, and relief of symptoms among people with syringomyelia, including those with Chiari I malformations.

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



NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

Last updated December 10, 2014