Syringomyelia

An excerpt from The Spinal Cord Injury Newsletter, Spring 1996
Copyright © 1996 by Medical News Publishing

Syringomyelia (SM) is a condition in which neurologic symptoms occur due to a syrinx, or fluid-filled cavity, that has formed within the spinal cord. The cavity may have expanded or elongated, causing pressure on the adjacent nervous tissue. This pressure can compromise the function of the surrounding nervous tissue and result in neurological symptoms. These can include new muscular weakness, such as reduced strength in neck, arm or hand muscles. New sensory loss, such as numbness or decreased temperature sensation may occur, as well as pain or spasticity at moderate to severe levels. Pain may occur as headache, limb or trunk pain, or sharp pain with coughing, and may be felt above, below or at the level of the injury. Both motor and sensory symptoms may be vague and difficult to describe or characterize. Decreased or increased levels of sweating may occur. Symptoms may occur on only one side of the body. SM can lead to new physical impairments, progressive paralysis, and serious affects on quality of life.

SYRINGOMYELIA AFTER SCI
There are several types of SM, including SM that occurs after spinal cord injury. Research studies have examined the location, size, shape and type of cavity in many different cases. These studies have found that a syrinx that forms after a spinal cord injury almost always appears at the site of the original injury. The syrinx may also extend several spinal segments above or below the level of injury. The shape of cavities, as shown by Magnetic Resonance Imaging (MRI), is also variable. There may be one oblong cavity or a large cavity may have several oblong compartments that are connected. Or there may be several separate cavities that have nerve tissue between them. The ends of the cavities may have a round, bulb-like shape or may stretch down to a thin strand.
How a syrinx forms and elongates is not understood. Medical researchers have proposed several explanations for syrinx development, but too little research has been conducted to provide strong support for any particular theory. The need for studies on cerebrospinal fluid dynamics and cyst formation was identified by a panel of experts brought together for a meeting on syringomyelia by the National Institute of Neurological Disorders & Stroke in June 1994.
Currently, SM is diagnosed following physical examination, history and progression of symptoms, and MRI of the spinal cord. The fluid-filled cavity usually shows up clearly on the MRI, revealing the size and location of the syrinx. It was generally believed that SM symptoms began long after the initial SCI, but recent studies suggest that syrinx-related symptoms may begin as early as two months after injury.
TREATMENT
The question of when and how to treat SM is not easy to answer, and different physicians take different approaches. Research studies do agree that it is important for the person with SM or suspected SM to find a physician who is knowledgeable about SM, including a neurosurgeon who has treated multiple cases of SM. A university hospital or rehabilitation clinic with an SCI unit is a reasonable place to start a physician search.
Usually, action is not taken when an MRI reveals a syrinx and there are no apparent symptoms. However, if an individual has a syrinx and has SM-related symptoms, a judgment must be made about whether the symptoms are serious enough to warrant the risks and expenses of surgical treatment. If the syrinx is quite large or is increasing in size, surgical treatment may be considered. In some cases, rehabilitation without surgery may be selected. For cases of post-traumatic SM that are severe enough to cause neurological symptoms, such as decreasing motor function or intractable pain, a surgical treatment may be the appropriate option.

RESEARCH
Research studies that have evaluated the outcomes of these surgical treatments have found mixed results in their effectiveness. Frequently, surgery leads to successful drainage of the syrinx and often the patient’s symptoms resolve or, at minimum, stabilize. However, not all patient’s symptoms are resolved; sometimes the surgery has little or no effect on pain, weakness or sensory loss. And, unfortunately, successful surgical treatment may result in only a temporary decrease in neurological symptoms. Months or years later, the symptoms may return.
Following any diagnosis or treatment of SM, follow-up and awareness of possible recurrence must be a priority for both patient and physician. This may include assessments of patient functioning over time and evaluation of future MRIs.
As physicians have become aware of the frequency of syrinx occurrence in persons with SCI and have struggled with the complexities of treating SM, researchers have become better focussed on the issues to be studied and the problems to be solved.

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