What Is a Chiari Malformation?
A Chiari malformation occurs when a deformity at the base of the skull, where the brain and spinal cord connect, causes part of the brain to protrude through a hole in the skull, affecting the flow of cerebrospinal fluid. Chiari malformations are defined by type — types I, II, III, IV and O — and, in most cases, are based on the length of the protrusion. However, ranking does not necessarily reflect the severity of the symptoms. Since Chiari malformations are rare, they can lead to misdiagnosis as multiple sclerosis, chronic fatigue syndrome, fibromyalgia or a spinal cord tumor.
In the vast majority of cases, people are born with Chiari malformations, which result when the brain stem and upper spinal cord fail to develop properly. The cause is not known.
The symptoms of Chiari malformations are highly variable and depend on the type of malformation affecting an individual. Some types, such as type I, are often asymptomatic, and types III and IV can be fatal.
When symptoms do occur, they often come and go. Some of the most common include:
Occipital headaches — This pain at the base of the skull, sometimes radiating to the neck and shoulders, is often made worse by coughing, sneezing or straining.
Problems affecting the eyes or ears — These can cause blurred or double vision, light sensitivity, pain behind the eyes, involuntary eye movement, dizziness, hearing loss or ringing in the ears.
Development of a syrinx — These fluid-filled cavities or cysts grow on the spinal cord, leading to a variety of additional symptoms, including muscle spasms, contractions and weakness, scoliosis, pain, and loss of bowel or bladder control.
Sleep disorders — People with Chiari malformations often experience sleep apnea and chronic fatigue.
Treatment is highly individualized and often involves specialists who can address specific symptoms. Treatment options include:
Medication and massage therapy — Noninvasive treatments can relieve pain associated with mild cases.
Surgery — While surgical options vary, the most common procedure is posterior fossa decompression, which involves removing bone at the base of the skull to relieve pressure on the brain stem.
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