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DEFINITION
Syringomyelia is a generic term
referring to a disorder in which a cyst or tubular
cavity forms within the spinal cord. This cyst, called
a syrinx, can expand and elongate over time, destroying
the spinal cord. Since the spinal cord connects the
brain to nerves in the extremities, this damage may
result 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. These
symptoms typically vary depending on the extent and,
often more critically, on the location of the syrinx
within the spinal cord.
Other, more common disorders share the early symptoms
of syringomyelia. In the past, this has made diagnosis
difficult. The advent of one diagnostic test, however,
called magnetic resonance imaging or MRI, has significantly
increased the number of syringomyelia cases diagnosed
in the beginning stages of the disorder.
Syringomyelia has a prevalence estimated at 8.4 cases
per 100,000 people, or about 21,000 Americans, with
symptoms usually beginning in young adulthood. Signs
of the disorder tend to develop slowly, although sudden
onset may occur with coughing, straining, or myelopathy.
If not treated surgically, syringomyelia can lead
to progressive weakness in the arms and legs, loss
of hand sensation, and chronic, severe pain.
SYMPTOMS
Syringomyelia causes a wide variety of neuropathic
symptoms due to damage of the spinal cord. Patients
may experience chronic pain, abnormal sensations and
loss of sensation particularly in the hands. Some
patients experience paralysis or paresis temporarily
or permanently. A syrinx may also cause disruptions
in the parasympathetic and sympathetic nervous systems,
leading to abnormal body temperature or sweating,
bowel control issues, or other problems. If the syrinx
is higher up in the spinal cord or affecting the brainstem
as in syringobulbia, vocal cord paralysis, ipsilateral
tongue wasting, trigeminal nerve sensory loss, and
other signs may occur. Rarely, bladder stones can
occur in the onset of weakness in the lower extremities.
Treatments
Physicians now use magnetic resonance imaging (MRI)
to diagnose syringomyelia. The MR imager takes pictures
of body structures, such as the brain and spinal cord,
in vivid detail. This test will show the syrinx in
the spine or any other conditions, such as the presence
of a tumor. MRI is safe, painless, and informative
and has greatly improved the diagnosis of syringomyelia.
The first step after diagnosis is finding a neurosurgeon
who is experienced in the treatment of syringomyelia.
Surgery is the only viable treatment for syringomyelia,
and a neurosurgeon is the only specialist qualified
to provide a fully informed recommendation. Not all
patients will advance to the stage where surgery is
needed. Evaluation of the condition is often difficult
because syringomyelia can remain stationary for long
periods of time, and in some cases progress rapidly.
Surgery results in stabilization or modest improvement
in symptoms for most patients. Delay in treatment
may result in irreversible spinal cord injury. Recurrence
of syringomyelia after surgery may make additional
operations necessary; these may not be completely
successful over the long term.
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