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Chiari Malformation is a birth defect that affects the cerebellum, causing balance, muscle control, and sensory perception issues. There are four types, with type IV being the most severe. Treatment options include medication and surgery.
A Chiari Malformation is a congenital malformation that causes defects in the cerebellum, the part of the brain responsible for balance, muscle control and sensory perception. There are four grades of Chiari malformation, with type I being the mildest and type IV being the most severe. Different treatment tools can be used to treat and manage a Chiari Malformation, which can allow the patient to live a relatively normal life, depending on the severity of the malformation.
This malformation was first described in 1800 by Hans Chiari. Another physician, Julius Arnold, also contributed to the body of knowledge about this birth defect, leading some people to refer to this condition as an Arnold-Chiari Malformation, in a desire to acknowledge both major early investigators. It is caused by a change in the shape of the skull that pushes the cerebellum down, forcing the cerebellum and brainstem into the spinal canal and restricting the flow of cerebrospinal fluid.
Patients with a type I Chiari malformation may experience symptoms so mild that they don’t even realize they have it. Type II and III patients have much more severe forms, which typically require treatment, while type IV patients may have an incompletely formed or developed brain, leading to very serious consequences. In all cases, a head scan can usually reveal the classic signs of a Chiari malformation, with the distorted shape of the brain clearly visible on an imaging study.
Chiari malformations can cause a variety of symptoms, including balance, muscle control, and vision problems. Some patients also experience headaches, random shooting pains, muscle weakness, dizziness, neck pain, difficulty swallowing, hearing problems, difficulty speaking, abnormal breathing, and spinal problems. Patients may also be prone to falls. Left untreated, a patient can develop paralysis or a buildup of fluid in the brain that could be extremely dangerous.
Specific medications can be used to help manage pain associated with a Chiari malformation. Additionally, surgical treatment can be used to relieve pressure on the cerebellum; usually several vertebrae or part of the skull can be removed to make room for the brain. Surgical treatment can also be used to address concerns about impaired brain and nervous system function caused by restricted circulation of cerebrospinal fluid. In patients who develop hydrocephalus, a shunt may be installed to drain excess fluid.
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