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Syringomyelia: Cavities in the Spinal Cord
Jessica Huls
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David, who enjoys going to his son's baseball games, has a
greatly progressed form of primary syringomyelia due to trauma
to the spine, which doctors link to rough games of his own
boyhood football. He brings along back support and cushions
for more comfortable seating. (Wes Newton)
Click image to enlarge.
When he was seventeen, in a relaxed Saturday afternoon football game with his buddies, David Newton dove for a long pass. He caught the ball like a superstar but landed hard on his elbow. He could tell something wasn’t moving right in his arm, so he headed to the hospital. David sat quietly in the examination room curiously glancing around at the equipment and anatomy charts until the doctor hurried in with the x-ray film. “Son, you’ve broken your elbow. In two places.” The doctor stared hard as David nodded. They hadn’t given him any painkiller yet, not even a single Tylenol. With a skeptical squint the doctor asked, “Aren’t you in excruciating pain?”
“No,” David shrugged, sort of surprised. “Not really.”
Now fifty-one, David stands in the kitchen of his country home in Nebraska running hot water into a sink full of dishes. He tilts forward as he walks, and limps a great deal due to a deteriorating knee. One arm hangs lower than the other. Severe scoliosis twists his spine into a hunch just between his shoulders.
“In retrospect, that lack of pain was the first sign of syringomyelia," he admits, "but I didn’t go in to have things checked out until about ten years later when I started to notice numbness and weakness in my hands.” He dunks a large thermometer into the dishwater before submerging his hands.
Syringomyelia, (from the Greek “syrinx” meaning tube and “myelus” meaning marrow) is a progressive disorder of the spinal cord that affects over an estimated 21,000 Americans and that is still not clearly understood. Syringomyelia develops when spinal fluid, which normally flows around the spinal cord, instead enters into the cord and creates a cavity known as a syrinx. Over time, the syrinx may expand and lengthen, severely damaging the nerve fibers at center of the spinal cord. Once this happens, a whole array of symptoms can occur, including muscle weakness, stiffness, difficulty walking, paralysis and quadriplegia. Syringomyelia usually causes chronic pain in the back while at the same time causing an inability to feel pain and temperature in other parts of the body, especially in the arms and hands.
Syringomyelia has at least two distinct causes. More than 70 percent of all cases are caused by an initial disorder called chiari malformation. Chiari malformation occurs when part of the brain (the cerebellar tonsils) protrudes down out of the bottom of the skull and crowds the top of the spinal cord. The direct link between chiari malformation and syringomyelia is still poorly understood but there is evidence that the extension of the cerebellar tonsils may cause a disturbance in the spinal fluid and thus cause a syrinx to form in the spinal cord.
Syringomyelia can also be caused by trauma to the spine. Today, as more people survive spinal cord injuries, more cases of post-traumatic syringomyelia are arising. As Dr. James Little, Associate Professor in the Department of Rehabilitation Medicine at the University of Washington mentions in his article published in Spinal Cord Injury Update, “Syringomyelia is an uncommon but disabling complication of SCI [spinal cord injury]. Although more than half of all people with SCI develop a cyst in the spinal cord at the injury site, only about four percent develop syringomyelia, in which the cyst fills with fluid and expands.” No one yet knows what causes a syrinx to form in one person and not in another. The prospects for people with syringomyelia related to chiari malformation are usually better than for those with syringomyelia caused by other complications. This is due in some part to the relief that a surgical correction of the protruding cerebellar tonsils can provide, allowing more normal function of the nervous system.
Once a cyst has begun to form, even coughing, sneezing and heavy lifting can create enough stress to cause an existing cyst or syrinx to enlarge, resulting in more damage. Primary syringomyelia can be caused by injuries due to sports, automobile accidents and even infections such as meningitis. And because the symptoms aren’t as prevalent at first, it can take years to diagnose syringomyelia, which unfortunately leads to poorer outcomes. Syringomyelia and chiari malformation were once considered very rare but doctors now feel that this was due to misdiagnosis and simple lack of knowledge of the conditions. Today, researchers report that 250,000 Americans have one or both of the disorders.
For David, as with most others affected by syringomyelia, the diagnostic process began with a neurological exam testing things like balance, strength and sensitivity in the hands. But ultimately, it is Magnetic Resonance Imaging (MRI) that answers any doubts and can visibly show a syrinx in the spinal cord. Recently, doctors have also begun using the Cine (as in cinema) MRI, which actually shows the flow of spinal fluid and whether or not it is somehow hindered.
David has a greatly progressed form of primary syringomyelia due to trauma to the spine which doctors link to rough games of boyhood football. Most likely, scar tissue began to clog the passageway where spinal fluid travels and a syrinx began to form.
This spinal fluid, more correctly called cerebrospinal fluid (CSF), is a clear, water-like substance that flows around the brain and spinal cord and acts as a cushion protecting the brain from injury and as a shock absorber for the spine. Every time the heart beats, blood passes through the choroid plexus, a set of filters in several parts of the brain, which separate the CSF from the blood. When a syrinx begins to form, it interferes with the flow of CSF and, due to the blockage, the fluid then begins to build up in the center of the spinal cord wreaking all sorts of havoc on the central nervous system.
“Fibers for feeling and movement run together in nerve highways called tracts,” according Dr. John Oro, Professor of Neurosurgery at the University of Missouri. “The spinal nerves bring sensory information, such as pain and temperature, into the spinal cord and send information out to muscles like on and off-ramps on a major highway.” Syringomyelia frequently starts at the center of the spinal cord and so the fibers located there, which carry information about pain and temperature, are often the first disturbed in people with the disorder.
David prefers not to spend much time dwelling on his disorder but in a way, it’s his job. By trade, he is a chiropractor and is still able to work part-time in Lincoln, Nebraska helping others with back pain and pain management. Though he is very limited by what his hands cannot do, David uses adjustment tools with large graspable handles to treat patients. He supplements with a less physical job at a medical supply store. He is often accused by friends and family of taking better care of others than of himself but he gets great satisfaction from his work knowing well that there is immense value in a caregiver who can relate.
David bumps into the table as he tries to sit in his chair, which is fixed with wedge and cylinder shaped foam pads for a more manageable sitting position. He has trouble fingering the pages of an information packet that he recently sent away for from the American Syringomyelia Alliance Project. “It says here that syringomyelia patients almost always have motor impairment,” he jokes sarcastically, as he unsuccessfully attempts to turn the page.
Other than motor impairment and loss of sensitivity to pain and temperature, warning signs of syringomyelia include severe headaches, muscle spasms, scoliosis, loss of bowel and bladder control, imbalance, and pain in the neck, shoulders, arms and legs. In the later years of the disorder, it often leads to loss of all normal function, resulting in life in a wheel chair or in bed.
David explains that most people with syringomyelia have some degree of insomnia due to muscle spasms and sleep apnea. He has figured out a way to get five solid hours of sleep each night by meticulously arranging pillows and cushions under his arms and legs, neck and certain places under his back.
David also suffers from what patients of the disorder call “brain fog.” David recalls a time when he visited on the phone for hours with a close friend who confided in him about a recent miscarriage he and his wife had experienced. The next time they talked David didn’t remember the past conversation and was just as surprised to hear of the miscarriage the second time. After hanging up, feeling terrible and thinking about it long and hard, things did start to come back to him. “It’s really embarrassing and trying. And names are almost a lost cause. I’ve just resorted to telling someone ‘Hey, I’m really sorry, I’m blanking on your name for some reason.’ Being honest is just easier than pretending.”
Syringomyelia affects people of all ages, races and genders. Since it is still uncertain how and why exactly the syrinx begins to form in the spinal cord, curing the problem is near impossible. Sometimes a person may form a syrinx with out ever having any symptoms at all.
The treatments that are available have had only limited success. A surgery to remove blockage and restore normal CSF flow is the most popular tactic but a recent study reported in Journal of Neurosurgery: Spine by Dr. Ulrich Batzdorf, a UCLA neurosurgery associate and expert on syringomyelia, shows that though many cases were stabilized, only fifteen out of sixty-four patients he operated on improved.
In his late twenties, David underwent surgery to place a shunt in his spine. Ideally, a shunt diverts fluid from the syrinx to another area of the body, often the abdomen, where it can safely be absorbed into the blood stream. In David’s case, he received a syringo-arachnoid shunt that ran from the center of the syrinx delivering the fluid to the surrounding layer of the cord where it is normally present. But several years later, the shunt failed and the syrinx filled and expanded again. This, unfortunately, is a common complication.
Generally, medications are the only road to relief for syringomyelia patients. Anti-inflammatories, sedatives, anti-spasmodics, diuretics, antidepressants and steroids are most commonly prescribed. Physical therapy and chronic pain specialists can be of great help. Consistent evaluations by a neurologist are recommended to identify other neurological disorders caused by syringomyelia.
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It is Magnetic Resonance Imaging (MRI) that answers any doubts
and can visibly show a syrinx in the spinal cord. Recently,
doctors have also begun using the Cine (as in cinema) MRI,
which actually shows the flow of spinal fluid and whether or
not it is somehow hindered. (Courtesy of the American
Syringomyelia Alliance Project, Inc.)
For David, what helps is just coming to terms with the fact that some things are out of one’s control. “In a way, it’s a sort of blessing to have a disorder that progresses slowly. It gives you time to prepare psychologically for the quality of life issues that will have to be faced as well as a shorter life expectancy.” And though chronic pain can be extremely alienating for a person, David keeps the clear perspective that he’s never the only one to suffer in the world. He is reminded by his patients and the people he helps fit to wheelchairs, that although days of assisted mobility are nearing, every moment he can walk on his own is a “gift.”
Syringomyelia is considered a terminal disease that causes a person to literally waste away. Ultimately, it is the complications that people eventually die from. Arms and legs become atrophied due to immobility; organs begin to fail because the body’s dramatic deformity causes them to over work. Stroke and aneurysm is a common occurrence for people in the later stages of syringomyelia.
But David leans on a strong sense of humor and a brawny sense of hope. “I have a feeling that in the next ten to twenty years, with all the stem cell research being done, syringomyelia may finally be successfully treated. Maybe stem cells will be inserted into the syrinx and will generate new and healthy spinal tissue. Who knows? And considering that some stem cell research is being done with fat tissue,” he says with a smirk, “the average American is sitting on a jackpot.” He laughs deeply and says with a nod and a song in his voice, “Yeah, I have hope.”
MORE INFO
To learn more about syringomyelia, visit:
--American Syringomyelia Alliance Project (ASAP) www.asap.org
--National Organization for Rare Disorders (NORD) www.rarediseases.org
--Brain Resources and Information Network (BRAIN) www.ninds.nih.gov
--National Spinal Cord Injury Association (NSCIA) www.spinalcord.org
NOTES
1. American Syringomyelia Alliance Project (www.asap.org)
a. Post-Traumatic Syringomyelia By Dr. James W. Little, (Originally published in the Fall 1998 issue of Spinal Cord Injury Update)
b. ASAP 2003 Conference Notebook, presentation notes by Dr. John Oro
2. National Institute of Neurological Disorders and Stroke (www.ninds.nih.gov)
3. Journal of Neurosurgery: Spine, December 2005, Volume 3, Number 6
4. National Organization for Rare Disorders (www.rarediseases.org)
5. Interviews with David Newton 2/15/2006, 4/11/2006, 5/5/2006
Letter to the Editor
A friend of mine just sent me this, and I wanted to thank you for putting it on your site. I have syringomyelia, as does the girl who sent this to me. I was diagnosed two years ago. Mine is idiopathic, meaning there is no reason for it. It came on while I had bronchitis. I am now 45. I am not to the extent of this man yet. But I went from being an active mother, wife, computer programmer, model, and actress, to being someone who is constantly in pain and wants to do nothing but sleep. I'm still all of the above, but only about half as much.
I just wanted to say thanks for publishing this. So many people have never heard of this--I know I never did until I was diagnosed with it. Maybe the more people who know about this, the more money can be donated for research. I, for one, am praying for a cure. I hope I never see the day when I have to be in a wheelchair, and I would love to see the day where I could live without pain.
Thanks again. --Glenna Reinhardt 1/9/07
Jessica Huls is a freelance essayist and science writer based
in Chicago Illinois. She has most recently had work published
in Fringe Magazine and Northern Virginia
Magazine.
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