Arnold Chiari Syndrome

Arnold Chiari Syndrome


What is Arnold Chiari Syndrome?

Arnold chiari syndrome is also known as prolapse of the cerebellum. It is a condition in which the lower end parts of the cerebellum move downward from the foramen magnum and form a hernia. This hernia also prevents the flow of cerebrospinal fluid and causes fluid accumulation in the brain.

The disease can sometimes be misdiagnosed, so factors such as the area where the pain develops, coughing and sneezing should be considered during the diagnosis.

Treatments vary according to the type of hernia.


There are 4 types of Arnold Chiari syndrome:

Type 1: Because the back of the head is small, it is the compression of the cerebellum. It can be seen in both adults and children. It is the most common type of Arnold Chiari syndrome and can cause a cyst inside the spinal cord.

Type 2: Occurs during birth and affects babies. It is seen together with spina bifida, which is a spinal cord anomaly. Spina bifida is when the spine does not close before birth and the spinal cord protrudes like a sac in the non-closure part.

Type 3: It is rare and occurs in infants. It is seen with a birth defect called encephalocele. An encephalocele is the formation of a fluid sac at the back of the baby's neck.

Type 4: occurs in infants. It occurs when the cerebellum does not develop properly.


Symptoms of Arnold Chiari Syndrome

Symptoms increase according to the progression of the disease, that is, the amount of sagging brain tissue. Symptoms vary according to the type of disease, the amount of pressure on the tissues and the amount of fluid that has accumulated.

Symptoms in adults:

  • Neck pain
  • Dizziness
  • Numbness in hands and feet
  • Difficulty swallowing
  • Hearing loss, ringing in the ears
  • Loss of sensation in the trunk and head
  • Curvature of the spine
  • Loss of balance
  • Symptoms in babies
  • Eating problem
  • Swallowing problem
  • Belching, vomiting
  • Irregular breathing
  • Slowing of development
  • Stigma
  • Weakness in the arms
  • Growth of the head
  • Epileptic seizures
  • Irritability


Causes of Arnold Chiari Syndrome

Arnold Chiari syndrome is sometimes caused by factors such as accident, infection (secondary), and sometimes it develops due to birth defects (primary).

The causes of primary brain prolapse are as follows:

  • Genetic mutations
  • Folic acid and vitamin deficiency during pregnancy
  • Infection and high fever during pregnancy
  • Smoking, alcohol and drug use during pregnancy
  • Poisoning or exposure to dangerous chemicals during pregnancy.



Treatment of the disease depends on the type of hernia and the severity of the symptoms. In some patients, drug therapy is applied, for some patients surgery is mandatory.