Syringomyelia occurs when a fluid-filled syrinx or cyst forms on the spinal cord. If the condition progresses, serious health complications can result. Though there are several possible causes of syringomyelia, the most common relate to Chiari malformations.
Symptoms of syringomyelia and their severity vary from one individual to another. Some people with syringomyelia experience no symptoms, but they can become so severe as to be disabling. Additionally, the condition may or may not progress slowly over time. Initially, muscle weakness, loss of sensitivity to temperature and pain, and loss of reflexes may occur in the hands, arms, shoulders, and back of the neck. Other symptoms include pain in the arms, neck, and back, muscle spasms and weakness in the legs, stiffness in the arms, shoulders, back, and legs, scoliosis (curvature of the spine), and bladder and bowel problems. Severe cases can result in paralysis in the arms and legs.
If syringomyelia progresses, affected individuals develop a range of symptoms such as Charcot joints. Chronic, progressive degeneration of the joints can result when there is damage to the nerve that supplies the joint. The joints become red and swollen and, without treatment, may deform. Some people with syringomyelia can develop problems with the autonomic nervous system, including a loss of bladder and bowel control, fluctuating blood pressure, and excessive sweating. Scoliosis or curvature of the spine may also occur, as does Horner's syndrome, in some cases. This rare condition results when one of the nerves that supplies the face and eyes is damaged. Horner's syndrome typically affects one side of the face. Signs of the condition include a droopy eyelid, decreased sweating on the affected side, reduced pupil size, and an inability to open the eye fully.
People of any age can develop syringomyelia, but the condition is most common in adults between the age of 20 and 40. Some research suggests the condition is slightly more common in males than females. Statistics show approximately 8.4 in 100,000 people are affected with syringomyelia in the United States.
The exact cause of syringomyelia is not yet known. Researchers believe disruption or obstruction of cerebrospinal fluid (CSF) is the most common trigger. CSF is a clear fluid that surrounds and protects the spinal cord and brain. Many conditions are associated with the development of syringomyelia, though Chiari malformation is most often linked. This condition causes brain tissue to descend into the spinal cord when part of the skull is misshapen or of below-average size, which places pressure on the brain and forces it down into the spinal cord.
Posttraumatic syringomyelia results from a spinal cord injury. The cyst can develop many years after the injury occurred. Meningitis, -- inflammation in the membranes surrounding the spinal cord and brain, arachnoiditis --inflammation in the arachnoid membrane, and spinal cord tumors are also associated with syringomyelia. Conditions present at birth, such as a tethered spinal cord, can also cause the condition.
After a thorough medical history and physical examination, if a doctor suspects syringomyelia, he will order one or more imaging tests:
If symptoms interfere with the patient's quality of life or the condition progresses quickly, a physician may recommend surgery, the goal of which is to restore the flow of cerebrospinal fluid and relieve pressure caused by the cyst. Doctors may turn to various surgical procedures depending on the specific cause of the condition. For instance, if syringomyelia is due to a Chiari malformation, surgery can enlarge the base of the skull and expand the brains covering. Sometimes, draining the cyst is the best course of action. A surgeon places a shunt consisting of a flexible tube between the cyst and another part of the body, such as the stomach. Fluid from the cyst drains out, relieving spinal pressure.
If a spinal abnormality interferes with the flow of cerebrospinal fluid, surgery can restore the proper flow of CSF. For instance, a doctor can surgically release a tethered spinal cord or remove obstructions such as spinal tumors. This action may restore the normal flow of CSF and enable the cyst to drain on its own.
Often, doctors will recommend lifestyle changes to manage symptoms before they consider surgery. Avoiding activities that put excessive pressure on the spine, as well as heavy lifting and straining, could alleviate some issues. Physical therapy may be beneficial as well. A physical therapist can create an exercise program that helps reduce stiffness, pain, fatigue, and muscle weakness.
Syringomyelia can recur, so follow-up examinations are essential. Often, the doctor will schedule regular exams and periodic MRIs or CT scans to ensure syringomyelia does not return. Additionally, even when treatment is successful, symptoms persist due to permanent damage.
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