NINDS Chiari Malformation Information Page: NINDS

Article title: NINDS Chiari Malformation Information Page: NINDS
Conditions: Chiari Malformation

What is Chiari Malformation?
Chiari malformation (also called Arnold-Chiari malformation) is a condition in which the cerebellum portion of the brain protrudes down into the spinal canal. Chiari malformation may be congenital, associated with other anomalies including myelomeningocele, syringomyelia, and spina bifida. Hydrocephalus (increased intracranial pressure) may also occur. Symptoms most often begin during infancy, although they may be delayed until adolescence or adulthood. Symptoms usually include vomiting, muscle weakness in the head and face, difficulty swallowing, and varying degrees of mental impairment. Paralysis of the arms and legs may also occur. As they grow older, adults and adolescents with Chiari malformation who previously were asymptomatic may show signs of progressive brain impairment, such as involuntary, rapid, downward eye movements. Other symptoms may include dizziness, headache, double vision, deafness, an impaired ability to coordinate movement, and episodes of acute pain in and around the eyes.

Is there any treatment?
Children with Chiari malformation may require surgery to repair an existing myelomeningocele. Hydrocephalus may be treated with surgical implantation of a shunt to relieve increased pressure on the brain. Some adults with Chiari malformation may benefit from surgery in which the opening in the back of the skull is enlarged to relieve intracranial pressure.

What is the prognosis?
Most patients who have surgery experience a reduction in their symptoms. Some patients may experience prolonged periods of relative stability. Infants with very severe malformations may have life-threatening complications.

What research is being done?
Basic research supported by the NINDS includes studies to understand how the brain and nervous system normally develop and function and how they are affected by disease and trauma. Studies of the developing brain and nervous system have opened promising new avenues of research and contribute to a greater understanding of congenital birth defects, including Chiari malformation.

Organizations

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
resourcecenter@modimes.org
http://www.modimes.org/
Tel: 914-428-7100 888-MODIMES (663-4637)
Fax: 914-428-8203

National Organization for Rare Disorders (NORD)
P.O. Box 8923
(100 Route 37)
New Fairfield, CT 06812-8923
orphan@rarediseases.org
http://www.rarediseases.org/
Tel: 203-746-6518 800-999-NORD (6673)
Fax: 203-746-6481

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

This fact sheet is in the public domain. You may copy it.Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892

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