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Chiari Malformation

Chiari Malformations (CM) are structural defects in the cerebellum (the part of the brain that controls balance). When the indented bony space at the lower rear of the skull is smaller than normal, the cerebellum and brainstem can be pushed downward. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid (the liquid that surrounds and protects the brain and spinal cord). There are three primary types of CM. The most common is Type I, which occurs during fetal development and may not cause symptoms and is often found by accident during an examination for another condition. Type II (also called Arnold-Chiari malformation) is usually accompanied by a myelomeningocele, a form of spina bifida that occurs when the spinal canal and backbone do not close before birth, causing the spinal cord to protrude through an opening in the back. This can cause partial or complete paralysis below the spinal opening. Type III includes a form of dysraphism with a portion of the cerebellum and/or brainstem pushing out through a defect in the back of the head or neck. Type III is very rare the most serious form of CM. It is associated with an early high mortality rate, or severe neurological deficits in patients that survive. Type IV is the most severe and rarest form of CM. In Type IV, the cerebellum fails to develop normally. There may be other associated malformations of the brain and brainstem. Most babies born with this malformation do not survive infancy.

Causes

There are different causes of Chiari Malformation. The most common is Primary CM which is caused by structural defects in the brain and spinal cord that occur during fetal development, whether caused by genetic mutations or lack of proper vitamins or nutrients in the mother's diet. Secondary CM can be caused later in life if spinal fluid is drained excessively from the lumbar or thoracic areas of the spine due to injury, infection or exposure to harmful substances.

Symptoms

Type I

Many people with Type I Chiari Malformation will have no symptoms and the condition may be discovered while doing other tests. If patients do have symptoms they may consists of any one or combination of the following:

  • Severe head and neck pain
  • Occipital headache felt at the base of the skull that is made worse by coughing, sneezing, etc.
  • Loss of pain and temperature sensation of upper torso and arms
  • Loss of muscle strength in the hands and arms
  • Drop attacks (collapsing to the ground due to muscle weakness)
  • Spasticity
  • Dizziness
  • Blurred Vision
  • Balance Problems
  • Sensitivity to bright lights

Type II

This type of malformation is characterized by downward displacement of the medulla, fourth ventricle, and cerebellum into the cervical spinal canal. This type occurs almost exclusively with patients with myelomeningocele (a form of spina bifida). Symptoms may include:

  • Changed pattern of breathing, including periods of apnea
  • Depressed gag reflex
  • Involuntary, rapid, downward eye movements
  • Loss of arm strength.

Type III

This type of malformation includes a form of spinal dysraphism with a portion of the cerebellum and/or brainstem pushing out through a defect in the back of the head and neck. Early operative closure of the defect is necessitated. Shunting is necessary to treat hydrocephalus which is commonly present. Additional severe birth defects are often present and will require extensive treatment.

Diagnosis

Because many people with CMs have no symptoms and their condition is discovered through coincidence, the physician will perform a physical exam and check memory, balance, cognition, touch, reflexes, sensations. One of the following may also be ordered.

Treatment

Some types of malformations are asymptomatic and do not require treatment as they do not interfere with a person's activities of daily living. In other cases where patients have symptoms such as pain, a physician may take a conservative approach and treat it with medication to ease the symptoms.

Surgery is the only treatment available to correct functional disturbances or halt the progression of damage to the central nervous system. Individuals who have surgery have seen a reduction in their symptoms. More than one surgery may be needed to treat the condition.

Type I and Type II may be treated with a local decompression of overlying bones to create more space for the cerebellum and to relieve pressure on the spinal column. Your surgeon will review every option with you and explain in detail what surgery may entail. Our experts at SCCNS have treated numerous Chiari Malformation patients with great success. We are here to answer any questions you may have.

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