What is Syringomyelia?
Syringomyelia is a chronic, progressive neurological condition and is sometimes associated with other neurological disorders such as Arnold-Chiari Malformation, osteoporosis, and scoliosis.
SM is characterised by a syrinx or pocket of fluid within the spinal cord. It results from an enlargement of the spinal cord due to excess fluid which builds up under pressure. The fluid is forced into the spinal cord causing debilitating effects.
The causes and symptoms of SM are numerous and no two cases are alike.
Common symptoms of SM can be motor impairment, severe pain, spacticity, numbness, weakness, and loss of sensitivity in the extremities.
SM is a rare condition which affects adults
and children. Currently, there are no statistics reported as to how many
Canadians are affected.
SM is a chronic progressive disease 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, and it affects both sensation and muscle power. It is sometimes spasmodic in it's deterioration, the precise clinical course can not be forecast, and it's 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 occur on coughing, sneezing, straining, or even the minute changes occurring with the arterial pulse are distributed and damped down within the system without 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 (eg. difficult birth), spinal injuries, or inflammation. It occurs in some paraplegics or quadriplegics, or in a few cases, spinal or brain tumours.
The result is that the pressure fluctuations create a fluid-filled cyst which gradually extends downward through the spinal cord and up into the brain, expanding over time. Because of the limited amount of room available in the cord, this expansion results in it's gradual destruction from the inside out, usually beginning with that part concerned with pain and temperature sensation in the upper limbs or the nerve fibres to the legs. In the end stages of a severely affected case, the cord may appear as a huge cyst with, in 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 (eg. burns) which are unnoticed until quite severe. This may progress to weakness of the arms, stiffness and weakness in 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 is 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 sufferer 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 become
depressed and require treatment for this.