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Arnold-Chiari Syndrome


About this topic


Arnold-Chiari syndrome is also called a Chiari malformation or CM. It is a defect in the lower part of the brain called the cerebellum. This part of the brain controls balance, coordination, and muscle movement. Sometimes, the brain stem is involved too. The brain stem controls things like breathing, heart rate, and blood pressure. ‚  
In most cases, there is a hole at the bottom of the skull for the spinal cord. The cerebellum and the brain stem are just above this hole, at the bottom of the skull. With a Chiari malformation, these two parts go down into the hole for the spinal cord. This can cause pressure to slow the flow of spinal fluid. Some people with this problem never have any signs. Others have serious problems. CM is grouped into 4 types: ‚  
  • Type I is the most common kind. With this, only the lower cerebellum goes into the hole. You may not have any signs. This is often found by chance in teenage years or as an adult during testing for other problems. It is the only type that can happen later in life.
  • Type II is also known as classic CM. Both the lower cerebellum and brainstem go into the hole. It is often connected to a kind of spina bifida. You may have partial or total paralysis.
  • Type III CM has the cerebellum, brainstem, and part of the brain into the hole. There are often severe nerve problems.
  • Type IV is very rare. The cerebellum is not fully developed.

What are the causes?


Most cases of CM are brain defects that happen before a baby is born. CM sometimes runs in families. There may be a gene that you get from your parent that makes you more likely to have this condition. You can also get a CM after surgery on your brain or spinal cord. ‚  

What can make this more likely to happen?


You are more likely to have a CM if you also have a condition like spina bifida or fluid on the brain. Having brain or back surgery raises your chances of having a CM. A babys risk is also higher if the mother did not get enough vitamins and minerals while pregnant. The baby's risk is also raised if the mother has an infection while pregnant or is exposed to harmful substances. ‚  

What are the main signs?


You may not have any signs with a CM. You may start to have signs later in life. Your signs may include: ‚  
  • Headache that is worse with coughing or straining
  • Neck pain
  • Muscle, bone, and nerve problems like:
    • Trouble with balance or walking
    • Problems with grasping small objects and hand coordination
    • Muscle weakness
    • Numbness or abnormal sensations in the arms and legs
    • Spinal curve problems
  • Problems with eyes and ears like:
    • Blurred eyesight
    • Rapid eye movements
    • Sensitive to light
    • Ringing or buzzing in ears
    • Hearing loss
  • Problems with mouth and throat like:
    • Gagging or trouble swallowing
    • Throwing up
    • Speech problems
  • Trouble sleeping or short times of stopped breathing while sleeping
  • Low mood

Signs in a baby may be a little different. They may include: ‚  
  • Fussy when being fed
  • Lots of drooling
  • Weak cry
  • Gagging or throwing up
  • Arm weakness
  • Stiff neck
  • Trouble breathing
  • Slow growth
  • Low weight gain

How does the doctor diagnose this health problem?


Your doctor will do an exam. Your doctor will check your reflexes, muscle movements, and strength. The doctor will also check your balance, coordination, and feeling. Your doctor may order: ‚  
  • X-rays
  • CT or MRI scan

How does the doctor treat this health problem?


If there are no signs, the doctors may not do anything. If you have signs, your doctor may suggest surgery to help ease them. Surgery may help to lower pressure at the base of your brain. Sometimes, the doctor will make the hole larger at the bottom of the skull. Ask your doctor if surgery would help you. ‚  

Are there other health problems to treat?


Sometimes, people with CM also have these problems that need treatment: ‚  
  • Hydrocephalus
  • Spina bifida
  • Cysts in the spinal canal
  • Spinal cord attaches to the bones of the spine. This is tethered cord syndrome.
  • Spine curves to the side or front
  • Behavior or thinking problems in children with CM.

What drugs may be needed?


The doctor may order drugs to: ‚  
  • Help with pain and swelling
  • Prevent or fight an infection
  • Ease other signs

What can be done to prevent this health problem?


There is nothing that can be done to prevent this problem. ‚  

Where can I learn more?


KidsHealth ‚  
http://kidshealth.org/parent/medical/brain/chiari.html ‚  
National Institute of Neurological Disorders and Stroke ‚  
http://www.ninds.nih.gov/disorders/chiari/detail_chiari.htm ‚  
NHS Choices ‚  
http://www.nhs.uk/conditions/chiari-malformation/Pages/Introduction.aspx ‚  

Consumer Information Use and Disclaimer


This information is not specific medical advice and does not replace information you receive from your health care provider. This is only a brief summary of general information. It does NOT include all information about conditions, illnesses, injuries, tests, procedures, treatments, therapies, discharge instructions or life-style choices that may apply to you. You must talk with your health care provider for complete information about your health and treatment options. This information should not be used to decide whether or not to accept your health care providers advice, instructions or recommendations. Only your health care provider has the knowledge and training to provide advice that is right for you. ‚  

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