SYRINGOMYELIA

Syringomyelia, (sir-ingo-my-eli-a) or SM is a progressive disorder of the nervous system.It is less common than multiple sclerosis, although it does have certain similarities. For instance it may show itself in early adult life, it affects both sensation and muscle power. It is sometimes spasmodic in its deterioration, the precise clinical course cannot be forecast and its cause or causes are largely unknown.

In this disease a cavity or syrinx develops within the spinal cord and sometimes the lower part of the brain. This cavity usually connects with the ventricles and subarachnoid space, fluid spaces within and around the brain. Normally the pressure changes in the fluid which can occur with coughing, sneezing or straining. Even minute changes occurring with the arterial pulse are distributed and damped down within the system with damage resulting.

In syringomyelia, however, there is often obstruction to pathways in the fluid spaces and this may occur as a result of congenital abnormalities of the brain, previous injury to the head, such as a difficult birth, or the possibility of a spinal injury, or inflammation. It occurs in some paraplegic or quadriplegics or in a few cases, spinal or brain tumors.

The result is that the pressure fluctuation creates a fluid-filled cyst which gradually extends downwards through the spinal cord and up into the brain, expanding with time. Because of the limited amount of room available in the cord this expansion results in its gradual destruction from the inside out, usually beginning with that part concerned with pain and temperature sensation in the upper limbs or the nerve fibers to the legs. In the end stages of a severely affected case, the cord may appear as a huge cyst with, in some places, only a tiny remnant of the original tissue as a thin superficial layer. This progression may take several decades and the disease has a tendency to arrest at any stage.

The earliest signs of the disease can occur in childhood up to the sixties, but is more common in early adult life. The onset may be painless injuries to the hands, such as burns, which are unnoticed until quite severe. This may progress to weakness of the arms, stiffness and weakness of the legs, deformities of the spine and involvement of the lower part of the brain, resulting in swallowing and breathing difficulties. Because the nerves supplying joints, especially the shoulders, are often damaged, degeneration of these joints occurs which in about half the cases, may be extremely painful. Sometimes the initial symptoms are of weakness or stiffness.

Immobility is a problem and can lead to pressure sores, thrombosis in the leg, veins, chest infections, joint stiffness and thinning of the bones. More disabling still may be the frustration that the suffer experiences with an active mind in a progressively less active body. Difficulty with breathing and swallowing can be worrying. Because of the progression in the course of the disease patients may require treatment for depression.

Various surgical operations have been devised to prevent enlargement of the syrinx. Perhaps the most important factor in the success of surgery is the age at which the disease is recognized. Progression may be prevented and some symptoms relieved, but little can be done where there is an expansive syrinx and a lot of cord destruction, as the substance of the cord is not capable of regeneration. Before surgery is considered, various pressure measurements may be taken and x-rays or Magnetic Resonance Imaging (MRI) scans performed to obtain information of relevance to the type of operation needed and for research purposes.

For additional information on Syringomyelia check out the following web sites
Canadian Syringomyelia Network
American Syringomyelia Alliance Project Inc.