Arnold-Chiari Syndrome

(Arnold-Chiari Malformation; Type II Chiari Malformation; Cerebellomedullary Malformation Syndrome)

Definition

Arnold-Chiari syndrome is a series of nervous system symptoms related to a deformity of the skull.This condition is often present at birth, but can also develop later in life. Some people with mild forms of this syndrome may never know they have the condition. For others it can be more severe. These severe forms will need treatment.

Causes

Arnold-Chiari syndrome is caused by a problem in the back of the skull. The skull should have an indented space in the back of the head. The rear lower part of the brain and the brainstem are in this space. In some people, this indented skull space does not develop well. This is called Chiari malformation. The space inside the skull is too small for the brain. As a result, the brain and brain stem are pushed downward. It blocks the flow of fluid from the brain to the spinal column. The problem with the skull develops before birth. It is not clear why it happens. In some cases, it occurs with a myelomeningocele, which is a form of spina bifida or syringomyelia, which is fluid cavites in the spinal cord.
Brain Stem and Lower Brain
GM00010 97870 brainstem.jpg
Copyright © Nucleus Medical Media, Inc.

Risk Factors

There are no known risk factors for Chiari malformation. There may be a genetic connection in some families.

Symptoms

Symptoms in infants are due to pressure on the lower part of the brain and brainstem. They may include:
  • Vomiting
  • Weakness
  • Paralysis of the limbs
  • Choking
  • Chronic cough or hoarseness
Symptoms in adolescents are usually milder and may include:
  • Lightheadedness
  • Fainting
  • Weakness in the legs
  • Headaches
  • Inability to hear or ringing in the ears
  • Eye problems such as difficulty seeing, eye pain, and rapid eye movement
  • Poor coordination
  • Uncontrolled shaking or trembling
  • Difficulty walking
  • Numbness or tingling in the arms or legs

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