What is Syringomyelia?
Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. This cyst, called a syrinx, can expand and elongate over time, destroying the spinal cord. The damage may result in loss of feeling, paralysis, weakness, and stiffness in the back, shoulders, and extremities.
Causes of Syringomyelia
It’s unclear exactly how and why syringomyelia happens. When it develops, cerebrospinal fluid — the fluid that surrounds, cushions and protects your brain and spinal cord — collects within the spinal cord itself, forming a fluid-filled cyst (syrinx).
Several conditions and diseases can lead to syringomyelia, including:
• Chiari malformation, a condition in which brain tissue protrudes into your spinal canal
• Meningitis, an inflammation of the membranes surrounding your brain and spinal cord
• Spinal cord tumor, which may interfere with the normal circulation of cerebrospinal fluid
• Conditions present at birth, such as a tethered spinal cord, a condition caused when tissue attached to your spinal cord limits its movement
• Spinal cord injury, which may cause symptoms months or even years after the initial injury
Symptoms of Syringomyelia
Syringomyelia symptoms usually develop slowly over time. If it is caused by protrusion of brain tissue into the spinal canal (Chiari malformation), symptoms generally may begin between ages 25 and 40.
In some cases, coughing or straining may trigger symptoms of syringomyelia, although neither causes syringomyelia.
The following early signs and symptoms of syringomyelia may affect the back of the neck, shoulders, arms and hands first:
• Muscle weakness and wasting (atrophy)
• Loss of reflexes
• Loss of sensitivity to pain and temperature
Other signs and symptoms of syringomyelia may include:
• Stiffness in the back, shoulders, arms and legs
• Pain in the neck, arms and back
• Bowel and bladder function problems
• Muscle weakness and spasms in the legs
• Spinal curvature (scoliosis)
How is Syringomyelia Diagnosed?
If your doctor suspects you have syringomyelia, you may be referred to a neurologist, a specialist in treating the nervous system.
To diagnose your condition, your neurologist will first take your complete medical history. A complete physical examination will also be performed. Be prepared to tell your neurologist about your symptoms and how long you’ve had them.
If your neurologist thinks you may have syringomyelia, they’ll order an MRI scan to look for a syrinx in your spinal cord. An MRI scan is the most reliable diagnostic tool for syringomyelia, and it’s considered the goldstandard for diagnosing the condition.
Treatment of Syringomyelia
Treatment depends on the progression of the disorder and whether you’re experiencing symptoms that disrupt your life. If you have no symptoms or mild symptoms, you may not need treatment. Your neurologist will monitor the progression of the disorder.
If your symptoms are negatively affecting your life, your neurologist will recommend medications or surgery.
Medications such as gabapentin (Neurontin) may help decrease the painful sensation of the shoulders and arms that frequently occur with syringomyelia.
The goal of surgery is to correct the underlying cause of the syrinx and relieve the pressure on your spinal cord. The type of surgery will depend on the cause of your syringomyelia.
If you have CM1, your surgeon may suggest surgery to expand the base of your skull and the covering of your brain. This will take pressure off your spinal cord and your brain. The normal flow of cerebrospinal fluid should be restored. For most people, this surgery resolves their syringomyelia.
If you have a tumor or bony growth that’s causing syringomyelia, removal of the growth frequently resolves the syringomyelia.
In some cases, your surgeon will use a small, flexible tube called a shunt to drain the syrinx. They’ll place the shunt in the syrinx to drain the excess fluid. Sometimes the surgeon can completely drain the syrinx during surgery. If that isn’t possible, the shunt will remain in place after your surgery.
After surgery, you may be prescribed a course of antibiotics to prevent complications from infection. Your doctor may also recommend physical therapy, which can help strengthen muscles in limbs that have progressive weakness.