What is Chiari?

Chiari Malformation is a debilitating neurological disorder which affects approximately 300,000 people in the United States alone. It does not discriminate against gender or age.

The cerebellum, the master of balance and coordination is compromised by it’s displacement in the brain therefore descending through the base of the skull into the spinal canal restricting spinal fluid from passing through and putting tremendous pressure in the brain, on the cerebellum and on the spinal cord.

There are 4 types of Chiari malformation all which have variations in symptoms by the severity of the disorder and the parts of the brain that protrude into the spinal canal. Chiari Malformation type 1 involves the extension of the cerebellar tonsils that are attached to the cerebellum and protrude into the foramen magnum. Normally only the spinal cord passes through the opening.

Depending on severity, common symptoms include neck pain, balance problems, muscle weakness, numbness in extremities, dizziness, vision problems, ringing in ears, hearing loss, head ache, difficulty swallowing, low muscle tone, lack of hand coordination and fine motor skills may be compromised.

Infants may gag on food, vomit, have arm weakness, a stiff neck, breathing problems, irritability when being fed, excessive drooling, weak cry, developmental delays and no weight gain.

Common conditions associated with CMs include:
Hydrocephalus – excessive build up of spinal fluid in the brain which blocks normal flow of this fluid, resulting in pressure within the head that can cause mental defects if left untreated, can be fatal. This can occur with any type of CM, but more commonly in type 2.

Spina Bifida is the incomplete development of the spinal cord to which the bones around the spinal cord do not properly form leaving the cord exposed and resulting in partial or complete paralysis.

Syringomyelia –a disorder in which the spinal fluid filled tubular cyst, or syrinx, forms within the spinal cords central canal. The growing syrinx destroys the center of the spinal cord, resulting in weakness, stiffness in the back, shoulders, arms or legs. Other symptoms include headaches and loss of the ability to feel hot or cold.

Tethered cord syndrome – occurs when the spinal cord attaches itself to the bony spine. The progressive disorder causes abnormal stretching of the spinal cord and resulting in permanent damage to the muscles and nerves in the lower body and legs.

CMs have several different causes. Primary CMs can be caused by structural defects in the brain and spinal cord during fetal development, caused by genetic mutations or lack of proper vitamins or nutrients in the maternal diet. Secondary CMs can also be caused later in life if the spinal fluid is drained excessively from the lumbar areas of the spine either due by injury, exposure to harmful substances or infection.

Surgical intervention is often necessary depending on severity and symptoms of Chiari Malformation and associated conditions. The prognosis is excellent for a vast majority of people.

  • gagging on food/difficulty swallowing
  • complaints of head pressure
  • moderate to severe headaches
  • numbness in limbs
  • vision problems
  • developmental delays  – Linked below is the  NYS Health Dept website that  can provide information with regards to evaluation of  your child’s overall development through their early intervention program.   You check can with your states health dept to see if they might offer this type of program http://www.health.state.ny.us/community/infants_children/early_intervention/county_eip.htm
  • vomiting
  • inability to gain weight
  • excessive drooling
  • stiff neck
  • breathing problems
  • irritability when being fed
  • hand coordination and fine motor skills may be affected.
  • weak tone
  • scoliosis