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| Syringomyelia |
Syringomyelia
Definition:
Syringomyelia (sear-IN-go-my-EEL-ya) is a disorder in which a cyst forms within the spinal cord. This cyst, called a syrinx,
expands and elongates over time, destroying the center of the spinal cord. Since the spinal cord connects the brain to nerves
in the extremities, this damage results in pain, weakness, and stiffness in the back, shoulders, arms, or legs. Other symptoms
may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Each patient experiences
a different combination of symptoms. Magnetic resonance imaging (MRI) has significantly increased the number of syringomyelia
cases diagnosed in the beginning stages of the disorder. Signs of the disorder tend to develop slowly, although sudden onset may
occur with coughing or straining. If not treated surgically, syringomyelia often leads to progressive weakness in the arms and legs,
loss of hand sensation, and chronic, severe pain. In most cases, the disorder is related to a congenital abnormality of the brain
called a Chiari 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.
Syringomyelia may occur as a complication of trauma, meningitis, hemorrhage, a tumor, or arachnoiditis. Symptoms may appear months
or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma.
Some cases of syringomyelia are familial, although this is rare
Symptoms:
The symptoms of SM are numerous and a person may have various combinations of different symptoms. Symptoms tend to develop slowly,
although sudden onset may occur with coughing and straining. Some common symptoms include: loss of sensitivity, especially to hot
and cold, muscle weakness and spasticity, motor impairment, loss of bowel and bladder control, as well as osteoporosis and scoliosis.
The majority of patients suffer from headaches and chronic pain.
Although Syringomyelia was first described over 400 years ago, very little research was performed and as a result little was known
about the disorder. Only recently, with the advent of MRI and the dramatic rise in diagnosed cases, have significant advancements been
made. In the past, SM was considered a disorder that always resulted in slow, progressive degeneration. Researchers now believe it
is possible that some patients may never progress.
Treatment:
Surgery is usually recommended for syringomyelia patients. Recurrence of syringomyelia after surgery may make additional operations
necessary; these may not be completely successful over the long term. In some patients it may be necessary to drain the syrinx, which
can be accomplished using a catheter, drainage tubes, and valves.
In the absence of symptoms, syringomyelia is usually not treated. 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 will be told
to avoid activities that involve straining.
Prognosis:
Symptoms usually begin between the ages of 25 and 40 and may worsen with straining or any activity that causes cerebrospinal fluid
pressure to fluctuate. Some patients, however, may have long periods of stability. Surgery results in stabilization or modest
improvement in symptoms for most patients. Delay in treatment may result in irreversible spinal cord injury.
Note: Portions of this text was obtained from The American Syringomyelia Alliance Project, Inc. (ASAP),
therefore this text is not copyright of Chiari Connection International (CCI) and remains copyright of The American Syringomyelia
Alliance Project, Inc. (ASAP).
Additional information can be found on their website:
ASAP
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