CHIARI MALFORMATION

The more common types of Chiari malformation are:

• Type 1
• Type 2

Although these types are less serious than the rarer pediatric form, type 3, signs and symptoms still can be life disrupting.

Chiari malformation type 1

In Chiari malformation type 1, signs and symptoms usually appear during late childhood or adulthood.

Headaches, often severe, are the classic symptom of Chiari malformation. They generally occur after sudden coughing, sneezing or straining. People with Chiari malformation type 1 can also experience:

• Neck pain
• Unsteady gait (problems with balance)
• Poor hand coordination (fine motor skills)
• Numbness and tingling of the hands and feet
• Dizziness
• Difficulty swallowing, sometimes accompanied by gagging, choking and vomiting
• Speech problems, such as hoarseness

Less often, people with Chiari malformation may experience:

• Ringing or buzzing in the ears (tinnitus)
• Weakness
• Slow heart rhythm
• Curvature of the spine (scoliosis) related to spinal cord impairment
• Breathing problems, such as central sleep apnea, which is when a person stops breathing during sleep

Chiari malformation type 2

In Chiari malformation type 2, a greater amount of tissue extends into the spinal canal compared with that in Chiari malformation type 1.

The signs and symptoms can include those related to a form of spina bifida called myelomeningocele that nearly always accompanies Chiari malformation type 2. In myelomeningocele, the backbone and the spinal canal don’t close properly before birth.

Signs and symptoms may include:

• Changes in breathing pattern
• Swallowing problems, such as gagging
• Quick downward eye movements
• Weakness in the arms

Chiari malformation type 2 is usually noted with ultrasound during pregnancy. It may also be diagnosed after birth or in early infancy.

Chiari malformation type 3

In the most severe type of the condition, Chiari malformation type 3, a portion of the lower back part of the brain (cerebellum) or the brainstem extends through an opening in the back of the skull. This form of Chiari malformation is diagnosed at birth or with an ultrasound during pregnancy.

This type of Chiari malformation has a higher mortality rate and may also cause neurological problems.