SymptomsBy Mayo Clinic staff
Doctors categorize Chiari malformation into four types, depending on the anatomy of the brain tissue that is displaced into the spinal canal, and whether developmental abnormalities of the brain or spine are present.
Many people with Chiari malformation have no signs or symptoms and don't need treatment. Their condition is detected only when tests are performed for unrelated disorders. However, depending on the type and severity, Chiari malformation can cause a number of problems.
The more common types of Chiari malformation are:
- Type I (adult)
- Type II (pediatric)
In Chiari malformation type I, signs and symptoms usually appear during late childhood or adulthood. Chiari II malformation is usually noted by ultrasound during pregnancy or at birth or early infancy. Although these types are less serious than the more rare pediatric forms, types III and IV, signs and symptoms still can be life disrupting.
Chiari malformation type I
Headaches, often severe, are the classic symptom of Chiari malformation. They're typically precipitated with sudden coughing, sneezing or straining. People with Chiari malformation type I can also experience:
- Neck pain (running down the shoulders at times)
- Unsteady gait (problems with balance)
- Poor hand coordination (fine motor skills)
- Numbness and tingling of the hands and feet
- Difficulty swallowing (sometimes accompanied by gagging, choking and vomiting)
- Vision problems (blurred or double vision)
- Slurred speech
Less often, people with Chiari malformation may experience:
- Ringing or buzzing in the ears (tinnitus)
- Poor bladder control
- Chest pain, in a band-like pattern around the chest
- Curvature of the spine (scoliosis) related to spinal cord impairment
- Abnormal breathing — specifically, sleep apnea, characterized by periods of breathing cessation during sleep
Chiari malformation type II
In Chiari malformation type II, a greater amount of tissue protrudes into the spinal canal compared with type I. The signs and symptoms can include those related to a form of spina bifida, called myelomeningocele, that always accompanies Chiari II malformation. In myelomeningocele, the backbone and the spinal canal have not closed properly before birth.
Chiari malformation type III
In one of the most severe types of the condition, Chiari malformation type III, a portion of the lower back part of the brain (cerebellum) or the brainstem extends through an abnormal opening in the back of the skull. This form of Chiari malformation is obvious at birth or by intrauterine ultrasound.
Chiari malformation type IV
In people with the even more severe Chiari malformation type IV, the brain itself has never developed normally. This form also is obvious at birth or by intrauterine ultrasound.
When to see a doctor
If you or your child has any of the signs and symptoms that may be associated with Chiari malformation, see your doctor for an evaluation.
Because many symptoms of Chiari malformation can also be associated with other disorders, a thorough medical evaluation is important. Head pain, for example, can be caused by migraines, sinus disease or a brain tumor, as well as Chiari malformation. Other signs and symptoms overlap with other conditions, such as multiple sclerosis.
- NINDS Chiari malformation information page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/chiari/chiari.htm. Accessed Sept. 23, 2010.
- Chiari malformation. American Association of Neurological Surgeons. http://www.aans.org/Patient%20Information/Conditions%20and%20Treatments/Chiari%20Malformation.aspx. Accessed Sept. 24, 2010.
- Syringomyelia. American Syringomyelia and Chiari Alliance Project. http://www.asap.org/index.php/disorders/syringomyelia/. Accessed Sept. 24, 2010.
- Developmental diseases of the nervous system. In: Ropper AH, et al. Adams and Victor's Principles of Neurology. 9th ed. New York, N.Y.: The McGraw-Hill Companies; 2009. http://www.accessmedicine.com/content.aspx?aID=3637082. Accessed Sept. 24, 2010.