Conquering Chiari Malformation
You have monstrous headaches. You suffer from muscle aches and chronic fatigue. You could have Chiari malformation.
Chiari malformation is a neurological condition in which the cerebellum—the bottom part of the brain—descends out of the skull and puts pressure on the brain and spinal cord.
This illness can be genetic, caused by structural defects that occur during fetal development, or acquired later in life.
The condition is rare, the prevalence in the general population is slightly less than 1 in 1,000.
Chiari malformation affects the cerebellum, the part of the brain that controls balance. Normally, the cerebellum and brain stem sit just above the opening at the base of the skull where the spinal cord and the brain connect. In Chiari malformation, the cerebellum and/or brain stem sit below this opening. When this occurs, the pressure that results may affect the functions controlled by that portion of the brain. It may also cause a disruption in the flow of cerebrospinal fluid that cushions the brain and spinal cord. The are four types of Chiari malformation with a fifth one under debate.
Type 1- is the most common form of Chiari malformation and may not cause symptoms until adolescence or adulthood, if at all. It may be present from birth, but it is also the only form that can be acquired (the result of another condition). Often, it is found during examination for another condition.
Type 2-this type is usually a consequence in children born with spina bifida (split spine defect). Also known as the Classic Chiari malformation, in this both, the cerebellum and the brain stem extend into the opening.
Type 3-this, despite being a rare case, is the most serious form, as it involves herniation and protrusion of the cerebellum and brain stem into the opening which has a great potential to cause severe neurological defects.
Type 4-being the rarest of all the other types, this type of malformation involves an incomplete/underdeveloped cerebellum and is sometimes associated with exposed parts of the skull.
Chiari malformation is also associated with other conditions such as syringomyelia, a cerebrospinal fluid-filled cyst that forms within the central canal of the spinal cord. As it enlarges, the cyst can cause pain and weakness and stiffness of the back, legs, shoulders or arms.
While birth defect in the spine (spina bifida) is a common reason, yet other conditions associated with Chiari malformation can be an excessive build up of the cerebrospinal fluid (hydrocephalus), disorder in the spine which is progressive that leads to the attachment of the spinal cord to the bony spine, or other spinal curvature deformities like scoliosis.
If symptoms are present, they may include neck pain, balance or vision problems, tinnitus, vomiting, insomnia, dizziness, difficulty swallowing, hearing loss, paresthesias, depression and headache worsened by straining or coughing. Symptoms may change depending on the pressure caused by built up cerebrospinal fluid.
Many patients have trouble getting a firm diagnosis because many of the symptoms resemble other illnesses. A definitive diagnosis only comes with an MRI. Some research indicates that a true diagnosis may take five years or more.
If the symptoms are not persistent and do not hinder the quality of life, then treatment may not be necessary. While in severe cases where medication provided to subside pain, do not provide relief and interfere with daily activities, the patient may require a surgical procedure.
While surgery remains the last resort to correct the functional outcomes or curb down the progression of the damage rate to the nervous system, the surgery aims to relieve the pressure on the brain and spine and re-establish the normal fluid circulation.
Speak to your health care provider about your options. If you have any general questions about Chiari malformation, you can visit asap.org for more information.
