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Syringomyelia
 
disorder in which a cyst 
  forms within the spinal cord.


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 syringomyelia

Do you have any of these symptoms?
Back pain; headaches; stiffness, weakness or pain in the back, shoulders, arms or legs; loss of the ability to feel extremes of hot or
 cold, especially in the hands?
You may have Syringomyelia (SM)

Table of Contents

  • What is syringomyelia?
  • What causes syringomyelia?
  • What are the different forms of syringomyelia?
  • How is syringomyelia diagnosed?
  • How is syringomyelia treated?
  • What research is being done?
  • Where can I go for more information?


    What is syringomyelia?

    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.

    Other, more common disorders share the early symptoms of syringomyelia. In the past, this has made diagnosis difficult. The advent of one outpatient test, however, called magnetic resonance imaging (MRI), has significantly increased the number of syringomyelia cases diagnosed in the beginning stages of the disorder.

    About 21,000 American men and women have syringomyelia, with symptoms usually beginning in young adulthood. 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.

     


    What causes syringomyelia?

    A watery, protective substance known as cerebrospinal fluid normally flows around the spinal cord and brain, transporting nutrients and waste products. It also serves to cushion the brain.

    A number of medical conditions can cause an obstruction in the normal flow of cerebrospinal fluid, redirecting it into the spinal cord itself. For reasons that are only now becoming clear, this results in syrinx formation. Cerebrospinal fluid fills the syrinx. Pressure differences along the spine cause the fluid to move within the cyst. Physicians believe that it is this continual movement of fluid that results in cyst growth and further damage to the spinal cord.

     


    What are the different forms of syringomyelia?

    Generally, there are two forms of syringomyelia. In most cases, the disorder is related to an abnormality of the brain called a Chiari I malformation, named after the physician who first characterized it. This anatomic abnormality 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. A syrinx may then develop in the cervical region of the spinal cord. Because of the relationship that was once thought to exist between the brain and spinal cord in this type of syringomyelia, physicians sometimes refer to it as communicating syringomyelia. Here, 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 suddenly. Some patients, however, may have long periods of stability. Some patients with this form of the disorder also have hydrocephalus, in which cerebrospinal fluid accumulates in the skull, or a condition called arachnoiditis, in which a covering of the spinal cord--the arachnoid membrane--is inflamed.

    The second major form of syringomyelia occurs as a complication of trauma, meningitis, hemorrhage, a tumor, or arachnoiditis. Here, the syrinx or cyst develops in a segment of the spinal cord damaged by one of these conditions. The syrinx then starts to expand. This is sometimes referred to as noncommunicating syringomyelia. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma.

    The primary symptom of post-traumatic syringomyelia is pain, which may spread upward from the site of injury. Symptoms, such as pain, numbness, weakness, and disruption in temperature sensation, may be limited to one side of the body. Syringomyelia can also adversely affect sweating, sexual function, and, later, bladder and bowel control.

    Some cases of syringomyelia are familial, although this is rare. In addition, one form of the disorder involves a part of the brain called the brainstem. The brainstem controls many of our vital functions, such as respiration and heartbeat. When syrinxes affect the brainstem, the condition is called syringobulbia.

     


    How is syringomyelia diagnosed?

    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 physician may order additional tests to help confirm the diagnosis. One of these is called electromyography (EMG), which measures muscle weakness. The doctor may also wish to test cerebrospinal fluid pressure levels and to analyze the cerebrospinal fluid by performing a lumbar puncture. In addition, computed tomography (CT) scans of a patient's head may reveal the presence of tumors and other abnormalities such as hydrocephalus.

    Like MRI and CT scans, another test, called a myelogram, takes x-ray-like pictures and requires a contrast medium or dye to do so. Since the introduction of MRI this test is rarely necessary to diagnose syringomyelia.

     


    How is syringomyelia treated?

    Surgery is usually recommended for syringomyelia patients. The main goal of surgery is to provide more space for the cerebellum (Chiari malformation) at the base of the skull and upper neck, without entering the brain or spinal cord. This results in flattening or disappearance of the primary cavity. If a tumor is causing syringomyelia, removal of the tumor is the treatment of choice and almost always eliminates the syrinx.

    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.

    In some patients it may be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. This system is also known as a shunt. Shunts are used in both the communicating and noncommunicating forms of the disorder. First, the surgeon must locate the syrinx. Then, the shunt is placed into it with the other end draining cerebrospinal fluid into a cavity, usually the abdomen. This type of shunt is called a ventriculoperitoneal shunt and is used in cases involving hydrocephalus. By draining syrinx fluid, a shunt can arrest the progression of symptoms and relieve pain, headache, and tightness. Without correction, symptoms generally continue.

    The decision to use a shunt requires extensive discussion between doctor and patient, as this procedure carries with it the risk of injury to the spinal cord, infection, blockage, or hemorrhage and may not necessarily work for all patients.

    In the case of trauma-related syringomyelia, the surgeon operates at the level of the initial injury. The cyst collapses at surgery but a tube or shunt is usually necessary to prevent re-expansion.

    Drugs have no curative value as a treatment for syringomyelia. Radiation is used rarely and is of little benefit except in the presence of a tumor. In these cases, it can halt the extension of a cavity and may help to alleviate pain.

    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.

     


    What research is being done?

    The precise causes of syringomyelia are still unknown. Scientists at the National Institute of Neurological Disorders and Stroke in Bethesda, Maryland, and at grantee institutions across the country continue to explore the mechanisms that lead to the formation of syrinxes in the spinal cord. For instance, Institute investigators have found that as the heart beats, the syrinx fluid is abruptly forced downward. They have also demonstrated the presence of a block to the free flow of cerebrospinal fluid that normally occurs in and out of the head during each heartbeat.

    Surgical techniques are also being refined by the neurosurgical research community. In one treatment approach currently being evaluated, neurosurgeons perform a decompressive procedure where the dura mater, a tough membrane covering the cerebellum and spinal cord, is enlarged with a graft. Like altering a suit of clothing, this procedure expands the area around the cerebellum and spinal cord, thus improving the flow of cerebrospinal fluid and eliminating the syrinx.

    It is also important to understand the role of birth defects in the development of hindbrain malformations that can lead to syringomyelia. Learning when these defects occur during the development of the fetus can help us understand this and similar disorders, and may lead to preventive treatment that can stop the formation of many birth abnormalities. Dietary supplements of folic acid during pregnancy have already been found to reduce the number of cases of certain birth defects.

    Diagnostic technology is another area for continued research. Already, MRI has enabled scientists to see conditions in the spine, including syringomyelia, even before symptoms appear. A new technology, known as dynamic MRI, allows investigators to view spinal fluid pulsating within the syrinx. CT scans allow physicians to see abnormalities in the brain, and other diagnostic tests have also improved greatly with the availability of new, non-toxic, contrast dyes. Patients can expect even better techniques to become available in the future from the research efforts of scientists today.

     


    Where can I go for more information?

    The National Institute of Neurological Disorders and Stroke conducts and supports a wide range of research on neurological disorders, including pain and painful disorders such as syringomyelia. For more information on this and other neurological disorders, or on the Institute and its research programs, contact the Institute's Brain Resources and Information Network (BRAIN) at:

    BRAIN
    P.O. Box 5801
    Bethesda, Maryland 20824
    (301) 496-5751
    (800) 352-9424
    www.ninds.nih.gov

    *The organization listed below provides printed information and assistance to syringomyelia patients and other interested individuals.

    American Syringomyelia Alliance Project, Inc.
    P.O. Box 1586
    Longview, Texas 75606-1586
    (903) 236-7079
    1-800-ASAP-282
    www.asap4sm.com/




 

 

 

 

 

  Hydromyelia or syringomyelia 
Hydromyelia or syringomyelia is the accumulation of fluid within the interior of the spinal cord. As the fluid accumulates it compresses the surrounding spinal cord tissue with symptoms developing. Usually the accumulation is in association with some abnormal state at the base of the brain or within the spinal canal housing the spinal cord. One group has classified syringomyelia according to the mechanism causing its formation.

or in association with a process which occurs after birth:
  1. inflammation at the outlets of the brain's interior fluid spaces
  2. tumors at the base of the brain
  3. cysts within the brain
  • Posttraumatic Syringomyelia: This develops after an injury to the spinal cord as the injured tissue is absorbed leaving a cavity. The cavity, for unclear reasons can then progressively enlarge
  • Syringomyelia in association with a inflammatory condition in the spinal fluid space (arachnoiditis)
  • Syringomyelia in association with spinal cord tumors.
  • Idiopathic Syringomyelia: Syringomyelia occurring in isolation without a clear reason



Treatment

 The first step in the treatment of a syrinx or hydromyelia is to determine why it exists. A vigorous effort is extended to insure that the cyst is not the secretion of a tumor hiding in the wall of the cavity. The apex of the spinal cord is imaged with the MR scanner to determine whether or not an Arnold Chiari Malformation is present. The adjacent bone of the spine is inspected to determine if a bony spur could have injured the spinal cord and thus caused the cyst.

Most surgeons would advocate exposing the spinal cord at the level of the cyst in order to drain it. This is done by a limited removal of bone overlying the cord ( a laminectomy) followed by opening of the investing tissue (the dura). While some would simply drain the cyst with a needle or make a small incision through the cord into the cyst (a myelotomy), we prefer to place a small tube through the myelotomy with one end in the cyst and the other in the fluid space surrounding the spinal cord to prevent the re-closure of the surgical opening. We prefer to make the opening below the neck so as to avoid injury to the spinal cord which would affect arm function.Rarely, a collapsed fluid cavity is encountered when the spinal cord is exposed. In this case the ultrasound is used to image the interior of the cord. If no cavity is seen, no myelotomy is performed.

The Hyman-Newman Institute for Neurology and Neurosurgery
Beth Israel Medical Center, Singer Division
New York, NY



 

 

 

 

 

 



 


 






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