Arnold Chiari Syndrome
- Article
- 2021-03-02
Chiari malformations (CMs) are structural defects in the base of the skull and cerebellum, the part of the brain that controls balance. When part of the cerebellum extends through the opening at the base of the skull, the cerebellum and brain stem can be pushed down. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid (CSF, the fluid that surrounds and protects the brain and spinal cord) and can cause a range of symptoms, including dizziness, muscle weakness, numbness, headaches, and hearing problems. balance and coordination. Symptoms may change for some individuals, depending on the build-up of CSF and the resulting pressure on tissue and nerves. CMs are classified based on the severity of the condition and the areas of the brain that protrude into the spinal canal. The most common is Type I, which may not cause symptoms and is often found accidentally during a search for another condition. Type II (also called classic CM and Arnold-Chiari malformation) is usually accompanied by a myelomeningocele - a form of spina bifida that occurs when the spinal canal and spine do not close before birth, forcing the spinal cord through an opening in the back. This can cause partial or complete paralysis under the opening of the spine. Symptoms of type III - the most severe form of CM - include those seen with type II, in addition to additional serious neurological defects. In CM Type IV, parts of the cerebellum are missing and parts of the spinal cord may be visible. Other conditions sometimes associated with CM include hydrocephalus, syringomyelia (a fluid-filled cyst in the spinal cord), and curvature of the spine.
Therapy
Medicines can relieve certain symptoms, such as pain, if any. In many cases, surgery is the only treatment available to improve or stabilize symptoms or to stop the progression of damage to the central nervous system. More than one surgery may be required to treat the condition. Surgery may include a procedure to create more space for the cerebellum or to remove some of the cerebellar tonsils that can reach through the cranial opening (the cerebellar tonsils have no recognized function and can be removed without causing known neurological problems ).
Some CMs have no noticeable symptoms and do not interfere with the person's daily activities.
Prognosis
Many people with Type I CM do not show symptoms and are unaware that they have the condition. The symptoms may change for some individuals, depending on the compression of the tissue and nerves and the build-up of crebrospinal fluid pressure. Many people with the more severe forms of CM who undergo surgery see a reduction in their symptoms and / or prolonged periods of relative stability, although paralysis is generally permanent.
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