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Atlantoaxial Instability in Children with Down Syndrome

​By: Marilyn J. Bull, MD, FAAP

Children with Down syndrome are at a slightly increased risk of developing compression of the spinal cord called atlantoaxial instability. This problem is caused by a combination of low muscle tone, loose ligaments and bony changes. The spinal cord can be pressed by the vertebrae bones in the neck and cause nerve damage.

Symptoms of nerve damage can occur at any time,​and there is no test or x-ray that can tell who is at risk. Here are some signs of atlantoaxial instability you can watch for and how the condition is diagnosed. 

Signs & symptoms of atlantoaxial instability

Contact your child's doctor immediately if you notice they have:

  • change in how they walk

  • change in how they use their arms/hands

  • change in bowel or bladder control

  • neck pain or stiffness, or their head stays tilted

  • new weakness or they become fatigued more easily

  • a decrease in their activity level or function

How is atlantoaxial instability diagnosed?

An x-ray of your child's neck in the neutral position will help diagnose atlantoaxial instability. If the x-ray is abnormal or symptoms continue, your child should be referred as soon as possible to a pediatric neurosurgeon or pediatric orthopedic surgeon experienced in managing atlantoaxial instability.

More information

About Dr. Bull

Marilyn J. Bull, MD, FAAP is lead author of the American Academy of Pediatrics Health Supervision Guidelines for Children and Adolescents with Down Syndrome.

Last Updated
American Academy of Pediatrics (Copyright © 2023)
The information contained on this Web site should not be used as a substitute for the medical care and advice of your pediatrician. There may be variations in treatment that your pediatrician may recommend based on individual facts and circumstances.
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