Chiari Malformation

(Kee-AR-ee)

The Chiari malformation is an abnormality in the lower part of the brain called the cerebellum. There are several different forms. The most common type is the Chiari I malformation (CM) which this article addresses. Less commonly, it may be known as Arnold-Chiari malformation, tonsillar herniation or tonsillar ectopia. Most cases of Chiari are congenital, meaning they are present from birth.

In normal anatomy, the cerebellar tonsils are located just above this line called the foramen magnum. But in an individual with Chiari, the tonsils hang below the line (herniate) into the spinal canal. The degree to which the tonsils extend can vary tremendously.

What are the symptoms?

The most common symptom of Chiari malformation is a headache, which begins at the back of the head (neck) and radiates upward. The pain is often made worse or can be brought on by coughing, sneezing or straining. These activities are known as valsalva maneuvers.

Visual problems such as nystagmus (involuntary eye movements), double or blurred vision may occur. Balance difficulties, vertigo and dizziness also may be present. Some people may have cranial nerve compression. This can result in apnea (cessation of breathing), gagging, swallowing difficulties, facial numbness or syncope (temporary loss of consciousness).

Patients may have muscle weakness, particularly in the upper extremities, coordination problems, and gait abnormalities. Imaging of the spine may reveal a fluid collection inside of the spinal cord, known as a syrinx. Some individuals may have hydrocephalus, a buildup of fluid in the ventricles of the brain.

What is syringomyelia?

(sear-IN-go-my-EEL-ya)

Syringomyelia, often referred to as SM, is a chronic disorder involving the spinal cord. The condition occurs when cerebrospinal fluid (normally found outside of the spinal cord and brain) enters the interior of the spinal cord, forming a cavity known as a syrinx.

This syrinx often expands and elongates over time, destroying the center of the spinal cord. As the nerve fibers inside the spinal cord are damaged, a wide variety of symptoms can occur, depending upon the size and location of the syrinx.

There are two major types of SM. In most cases it is related to a congenital malformation involving the hindbrain (cerebellum) called a Chiari I Malformation. This malformation occurs during fetal development and is characterized by downward displacement of the lower part of the brain (cerebellar tonsils) beneath the foramen magnum, into the cervical spinal canal. This displacement blocks the normal flow of cerebrospinal fluid. When normal flow is obstructed, a syrinx can form in the spinal cord. Not all patients with Chiari Malformations will develop a syrinx, however.

SM can also occur as a complication of trauma, meningitis, tumor, arachnoiditis, or a tethered spinal cord. In these cases the syrinx forms in the section of the spinal cord damaged by these conditions. As more people are surviving spinal cord injuries, more cases of post-traumatic SM are being diagnosed as the syrinx can form years after the trauma.

What are the symptoms?

Symptoms develop slowly over time, but can come on suddenly after a fall or minor trauma. Sensation may be affected first. Some common symptoms include:

•Loss of sensitivity to hot and cold

•Numbness and tingling

•Bowel and bladder function may be affected

•Scoliosis

•Pain

•Muscle weakness

•Spasticity

•Paralysis (in severe cases, quadriplegia)

Scoliosis may be the only symptom in children. Often people with scoliosis undergo spinal MR imaging because of an atypical left-sided thoracic curve.

Many individuals suffer from chronic pain and some will develop neuropathic pain syndromes. This type of pain is difficult to treat. A large percentage of people have headaches which can be severe.

The Winter Holiday Marketplace will be holding a HUGE raffle and small carnival to help raise money for this organization and some of the kiddos will be there as well selling some awesome items they have made.

Come on down and support them too! It will be fun for the whole family.

The American Syringomyelia & Chiari Alliance Project, Inc. (ASAP) is a non-profit, 501(c)(3), member supported organization, chartered in May 1988.

ASAP funds educational, support and research programs. ASAP works toward fulfilling its mission to improve the lives of persons affected by syringomyelia, Chiari malformation and related disorders while we find the cure.

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