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What is Chiari Malformation?

Chiari Malformation is a structural defect in the cerebellum and the brainstem which control the balance and most of the involuntary functions of the body. The tissue from the cerebellum protrudes into the spinal canal because of the unusually small or deformed skull.

  The pressure on the cerebellum that is produced as a result of this malformation could prevent the cerebrospinal fluid from flowing normally. Hans Chiari is the name of the pathologist from Austria, who first described the condition and his colleague, Dr. Julius Arnold also contributed to the definition of this condition.

Chiari malformations are usually classified into  Type I and Type II. Type I progresses during the growing period of the person when the skull is also enlarging. So the full symptoms of this condition are usually seen only in adults. The Type II Chiari Malformation, is also called the Arnold-Chiari Malformation. It is congenital in nature and is already present in the baby when it is born. In many babies with the type II condition, it is often observed that a form of Spina Bifida known as myelomeningocele is also present. This is when the spinal canal and the vertebral column does not close completely before birth. Some scientists also include two more types of this condition as Types III and IV. While Type III is linked to occipital encephalocele, Type IV exhibits a condition where there is a lack of cerebellar development.

Symptoms of Chiari Malformation

Chiari malformations are quite rare and as a result the diagnosis may not be easy. The blockage of the cerebrospinal fluid can produce a variety of symptoms. These could be balance problems like dizziness, muscle debility, lack of sensation, vision problems, headache, and problems with balance and coordination. Babies with Type II  may be completely or partially paralysed in the lower extremities. Type III is the most serious form exhibiting several kinds of severe neurological defects. Hydrocephalus, syringomyelia, and spinal curvature are also considered by some to be types of Chiari Malformations.

Treatment for Chiari Malformation

The treatment options for Chiari Malformation depend on the severity of the condition. Pain relieving and anti-inflammatory medications for symptoms such as headaches are normally prescribed. Surgical intervention to reduce the pressure and restore the normal flow of the cerebrospinal fluid is a common approach in many hospitals.

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