My experience with Chiari (type I)

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It is my understanding that Chiari is a malformation (usually a birth defect) of

the spinal cord and skull which causes inflammation of the lower part of the

cerebellum and/or the upper part of the spinal cord due to ?rubbing? or friction

of nerves in contact with the skull or spine.

The inflammation often causes swelling which is called Syringomyelia = A

condition where a 'pocket' filled with fluid forms in the spinal cord . This can

cause permanent damage to the spine.

Syrinx = The fluid filled 'pocket' in the spine of someone with Syringomyelia

(cyst)

Generally, there are two forms of syringomyelia. In most cases, the disorder is

related to an abnormality of the brain called a Chiari I malformation, named

after the physician who first characterized it. This anatomic abnormality causes

the lower part of the cerebellum to protrude from its normal location in the

back of the head into the cervical or neck portion of the spinal canal. A syrinx

may then develop in the cervical region of the spinal cord. Because of the

relationship that was once thought to exist between the brain and spinal cord in

this type of syringomyelia, physicians sometimes refer to it as communicating

syringomyelia. Here, symptoms usually begin between the ages of 25 and 40 and

may worsen with straining or any activity that causes cerebrospinal fluid

pressure to fluctuate suddenly. Some patients, however, may have long periods of

stability. Some patients with this form of the disorder also have hydrocephalus,

in which cerebrospinal fluid accumulates in the skull, or a condition called

arachnoiditis, in which a covering of the spinal cord--the arachnoid

membrane--is inflamed.

Surgery is the only way to try to 'cure' the condition, however it is not 100%

successful and should never be taken lightly. The surgery requires the removal

of bone from the base of the skull and from the upper vertebrae. This relieves

the pressure on the nerves (cerebellum) and hopefully the inflammation (Syrinx)

will go away in time.

The birth defect which causes it is (again my understanding of the doctor?s

description in 1998) was that in normal human growth, the spine closes around

the spinal cord leaving no room for the cerebellar tonsils to swell into the

cavity. But in cases of Chiari the spine does not completely encircle the

spinal cord and allows it to rub against the skull.

At least that is how it was explained to us many years ago. Note too, that a

Syrinx can ALSO be caused by trauma or other injury anywhere in the spinal cord.

My daughter?s Chiari type I was diagnosed by a MRI and the cyst (Syrinx) is

obvious on the MRI, but as she was not having extreme symptoms, they recommended

that she not have the operation. That was four years ago and the cyst has not

grown.

The parts in italics above are taken from websites about Chiari and

Syringomyelia and the NIH website.

Take care, Bill Werre