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Chiari Malformation Rare Neurological Disorder

Chiari malformations (CMs) are a rare structural disorder which affects the cerebellum, the part of the brain which controls large motor activity, coordination, and fine motor function. The cerebellum also plays a role in cognitive function in terms of attention and language. The cerebellum also regulates fear and pleasure response. Chiari malformations affect equilibrium, fine movement, and greatly impact persons living with the disorder physically, sociologically, and emotionally. In Chiari Malformation, the lower portion of person's brain, known as the cerebellum, slips into the bony space at the base of the skull which is malformed and generally smaller than people without CM's. The pressure on the cerebellum can block cerebrospinal fluid, which is the bodies lubricant which protects the brain and spinal cord, and causes often unmanageable pain, muscle weakness, vision problems, headache, and balance and coordination impairment. It is unknown if Chiari malformation has a genetic basis or hereditary influence.

Chiari Malformations (CM's) typically fall within one of four categories. Type I CM's often impacts the patient very little, as it usually does not cause symptoms and is frequently found accidentally while a patient is treated for other disorders. Type II CM's are usually more serious and cause myleomendocele, a form of spina bifida caused when the spinal canal and back bone do not fuse in utero, a cavity or cyst forms within the spinal column, and the spinal cord protrudes through an opening in the back. Type II CM's can cause partial or complete paralysis. Type III is by far the most serious form of CM. Type III CM has severe neurological impact on the person living with the disorder with a wide range of symptoms including (but not limited to) dizziness, nausea, gastrointestinal disorders, frequent and painful headaches, loss of equilibrium, fainting, and sometimes even hydrocephalus, syringomyelia, spinal curvature, and complete paralysis. Type IV CM's are usually detected at birth or by intrauterine ultrasound during pregnancy. Infants suffering with Type IV CM's have a brain which never normally developed. Babies born with Type IV CM's generally do not live past the first three months of life.

There is no known treatment for CM's though medications can ease a person's symptoms. Surgery is often a treatment option to decrease "functional disturbances or halt the progression of damage to the central nervous system." Doctors can usually only offer a reduction of a person's symptoms in terms of treatment options and rarely prolonged periods of relative "stability." If paralysis does occur due to Chiari Malformation, it is typically permanent. More funding and research into this disorder must be done into the treatment and perhaps cure of those surviving with Chiari Malformation.

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