Syringomyelia is a condition in which a cyst forms within the spinal cord. This cyst, called a syrinx, expands and lengthens over time, destroying the center of the spinal cord.
Because syringomyelia can occur in association with other conditions, estimates of the number of Americans with syringomyelia vary widely, but a conservative estimate is that about 40,000 people in the United States are affected, with symptoms usually beginning in young adulthood.
In most cases, syringomyelia is related to a congenital abnormality of the brain called a Chiari type I malformation. This malformation occurs during the development of the fetus and 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. This form of syringomyelia is called communicating syringomyelia.
Syringomyelia can also occur as a complication of:
When it occurs as a result of one of these conditions, it is called non-communicating syringomyelia.
Symptoms of Syringomyelia
Symptoms usually begin in young adulthood. The symptoms tend to develop slowly, although sudden onset may occur with coughing or straining.
When the spinal cord is damaged, symptoms can include:
- Chronic severe pain, weakness, and stiffness in the back, shoulders, arms, or legs
- Headaches
- Loss of ability to feel extremes of hot or cold, especially in the hands
- Loss of bowel or bladder function
- Sweating problems
- Loss of sexual function.
However, these possible syringomyelia symptoms are not sure signs of syringomyelia. Other problems can also cause these symptoms. Anyone with these symptoms should see a doctor so that the problem can be diagnosed and treated as quickly as possible.
Diagnosing syringomyelia in the past had been difficult, because other conditions share similar symptoms. But the widespread availability of an outpatient imaging procedure called magnetic resonance imaging (MRI) has significantly increased the number of cases diagnosed in the beginning stages of the disorder.
Other tests that may be used to make a diagnosis include:
- Electromyography (EMG)
- Lumbar puncture
- Computed tomography (CT scan)
- Myelogram.
(Click Diagnosing Syringomyelia for more detailed information.)
How Is Syringomyelia Treated?
Treatment options depend on several factors, including:
- Presence and severity of symptoms
- Form of syringomyelia.
In the absence of
syringomyelia symptoms, the condition is usually not treated. During this period of "watchful waiting," patients are regularly monitored with an MRI and physical exam.
In addition, a physician may recommend not treating the condition in patients of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many patients are told to avoid activities that involve straining.
For patients with symptoms, the syringomyelia treatment of choice is surgery.
Prognosis for Syringomyelia
Many factors will determine how quickly or slowly the disease progresses and, therefore, the prognosis for syringomyelia.
Some people have no symptoms and a fairly stable syrinx. Others may have symptoms that worsen with straining or with any activity that causes cerebrospinal fluid (CSF) pressure to fluctuate. Others may have long periods of stability. For some, syringomyelia may progress rapidly.
For people with syringomyelia symptoms, surgery may result in stabilization or modest improvement in symptoms. For these patients, delay in syringomyelia treatment may result in irreversible spinal cord injury. Recurrence of syringomyelia after surgery may make additional operations necessary. However, these may not be completely successful in the long term.
Because of the variability of syringomyelia progression and, therefore, the prognosis, it is best to talk with a doctor experienced in treating syringomyelia.
