Chiari malformation type 1

What causes Chiari malformation type 1?

Primary or congenital Chiari malformations are caused by structural defects in the brain and spinal cord that occur during fetal development. The underlying cause of the structural defects are not completely understood, but may involve genetic mutations or lack of proper vitamins or nutrients in the maternal diet.

Less frequently, Chiari malformation type 1 is acquired after birth. Causes of acquired Chiari malformation type 1 involve the excessive draining of spinal fluid from the lumbar or thoracic areas of the spine as a result of injury, exposure to harmful substances, or infection. Click here to view a diagram of the spine.

Last updated on 05-01-20

What is the prognosis for someone diagnosed with Chiari malformation type 1?

The prognosis for someone diagnosed with Chiari malformation type 1 is often unknown. Many people with this condition do not have any symptoms, and it is not possible to predict if symptoms will develop in the future. Other people with Chiari malformation may experience dizziness, muscle weakness, numbness, vision problems, headache, or problems with balance and coordination. In these people, it is not always known if symptoms will get worse over time. It is important for people with Chiari malformation type 1 to receive regular medical care so they can be monitored by a physician for any new symptoms.

There are some treatments that may help reduce or eliminate the symptoms of Chiari malformation type 1. Medications may ease certain symptoms, such as pain. Surgery is another treatment option, but it is typically reserved for people with severe symptoms. Many patients who have had surgery see a reduction in their symptoms and/or prolonged periods of relative stability.

Last updated on 05-01-20

How might Chiari malformation type 1 be treated?

Some individuals with Chiari malformation type 1 do not have symptoms and do not require treatment. People who have mild symptoms, without syringomyelia, can typically be treated conservatively. Mild neck pain and headaches can usually be treated with pain medications, muscle relaxants, and the occasional use of a soft collar.

People with more severe symptoms may need surgery. Surgery is the only treatment available to correct functional disturbances or stop the progression of damage to the central nervous system. The goals of surgical treatment are decompression of the point where the skull meets the spine (the cervicomedullary junction) and restoration of normal flow of cerebrospinal fluid in the region of the foramen magnum (the hole in the bottom of the skull where the spinal cord passes to connect to the brain). Prognosis after surgery for the condition is generally good and typically depends on the extent of neurological deficits that were present before the surgery. Most people have a reduction of symptoms and/or prolonged periods of relative stability. More than one surgery may be needed to treat the condition.

Last updated on 05-01-20

Where To Start

Bobby Jones Chiari & Syringomyelia Foundation

The Bobby Jones Chiari & Syringomyelia Foundation offers information on Chiari malformation type 1

Last updated on 04-27-20

Name: American Syringomyelia & Chiari Alliance Project ASAP PO Box 1586
Longview, TX, 75606-1586 , United States
Phone: 903-236-7079 Toll Free: 800-272-7282 Fax : 903-757-7456 Email: Url:
Name: Bobby Jones Chiari & Syringomyelia Foundation Bobby Jones CSF c/o Dorothy Poppe 29 Crest Loop
Staten Island, NY, 10312, United States
Phone: +1-718-966-2593 Fax : +1-718-966-2593 (please call first) Email: Url:
Name: Worldwide Syringomyelia & Chiari Task Force Inc. PO Box 491975
Lawrenceville, GA, 30049,
Phone: +1-914-510-CURE (2873) Email: Url:

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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