Chiari I malformations arise when a part of the brain, cerebellar tonsils, extends down into the opening at the base of the skull where the spinal cord exits (foramen magnum). The presence of the tonsils within the foramen magnum can result in abnormal spinal fluid flow and create direct pressure on the brainstem. These malformations usually occur without any known cause.
The most common symptom is headache, which is occurs in the back of the head and the neck, and is increased by coughing or sneezing. Chiari I is usually seen in adults. Sometimes the malformation can lead to cyst formation within the spinal cord. These cysts can cause weakness and numbness in the hands and arms. Other symptoms include dizziness, incoordination, double vision, swallowing difficulties and involuntary eye movements.
A thorough history and physical, including an extensive neurological examination, is the first step in diagnosing Chiari malformation. MRI studies of the brain and cervical spine provide the most accurate method of diagnosing Chiari malformation and/or cyst formation (hydromyelia).
When symptoms are mild, medical treatment of headaches is often all that is required. For patients with severe pain or neurological deficits due to compression or cyst formation, surgery is usually indicated. Surgical treatment involves removing a portion of the skull and upper most cervical lamina. The surgery is performed to create more room at the foramen magnum and take pressure off of the brainstem.
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