Syringomyelia

Syringomyelia (sih-ring-go-my-E-lee-uh) is the development of a fluid-filled cyst (syrinx) within your spinal cord. Over time, the cyst can enlarge, damaging your spinal cord and causing pain, weakness and stiffness, among other symptoms.

Syringomyelia has several possible causes, though the majority of cases are associated with a condition in which brain tissue protrudes into your spinal canal (Chiari malformation).

Other causes of syringomyelia include spinal cord tumors, spinal cord injuries and damage caused by inflammation around your spinal cord.

If syringomyelia doesn't cause problems, monitoring the condition might be all that's necessary. But if you're bothered by symptoms, you might need surgery.

Symptoms

Syringomyelia symptoms usually develop slowly over time. If your syringomyelia is caused by protrusion of brain tissue into your spinal canal (Chiari malformation), symptoms generally begin between ages 25 and 40.

In some cases, coughing or straining can trigger symptoms of syringomyelia, although neither causes syringomyelia.

Signs and symptoms of syringomyelia, which might affect your back, shoulders, arms or legs, can include:

• Muscle weakness and wasting (atrophy)
• Loss of reflexes
• Loss of sensitivity to pain and temperature
• Headaches
• Stiffness in your back, shoulders, arms and legs
• Pain in your neck, arms and back
• Spinal curvature (scoliosis)

When to see a doctor

If you have any of the signs or symptoms associated with syringomyelia, see your doctor.

If you've had a spinal cord injury, watch for signs and symptoms of syringomyelia. Months to years can pass after an injury before syringomyelia develops. Make sure your doctor knows you had a spinal cord injury.

Causes

It's unclear 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 can 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 can cause symptoms months or years later

Complications

In some people, syringomyelia can progress and lead to serious complications. Others have no symptoms.

Possible complications as a syrinx enlarges or if it damages nerves within your spinal cord include:

• An abnormal curve of your spine (scoliosis)
• Chronic pain as a result of spinal cord damage to the spinal cord
• Motor difficulties, such as weakness and stiffness in your leg muscles that can affect your walking
• Paralysis

Diagnosis

Your doctor will ask about your medical history and do a complete physical examination. In some cases, syringomyelia might be discovered incidentally during a spine MRI or CT scan conducted for other reasons.

If your doctor suspects syringomyelia, you'll likely undergo tests such as:

• MRI. An MRI of your spine and spinal cord is the most reliable tool for diagnosing syringomyelia.

  An MRI uses radio waves and a strong magnetic field to produce detailed images of your spine and spinal cord. If a syrinx has developed within your spinal cord, your doctor will be able to view it on the MRI.

  In some cases, a specialist will inject a dye into a blood vessel in your groin, which travels through blood vessels to your spine and reveals tumors or other abnormalities.

  You might have repeated MRI scans over time to monitor the progression of syringomyelia.

• CT scan. A CT scan uses a series of X-rays to create a detailed view of your spine and spinal cord. It can reveal tumors or other spine conditions.

Treatment

Treatment for syringomyelia depends on the severity and progression of your signs and symptoms.

Monitoring

If syringomyelia isn't causing signs or symptoms, monitoring with periodic MRI and neurological exams might be all you need.

Surgery

If syringomyelia is causing signs and symptoms that interfere with your life, or if signs and symptoms rapidly worsen, your doctor will likely recommend surgery.

The goal of surgery is to remove the pressure the syrinx places on your spinal cord and to restore the normal flow of cerebrospinal fluid. This can help improve your symptoms and nervous system function. The type of surgery you'll need depends on the cause of syringomyelia.

To reduce pressure on your brain and spinal cord, surgery options include:

• Treating Chiari malformation. If syringomyelia is caused by Chiari malformation, surgery might involve removing a small section of bone at the back of your skull. This surgery can reduce pressure on your brain and spinal cord, restore the normal flow of cerebrospinal fluid, and might improve or resolve syringomyelia.
• Draining the syrinx. Your doctor will surgically insert a drainage system, called a shunt. It consists of a flexible tube that keeps fluid from the syrinx flowing in the desired direction. One end of the tubing is placed in the syrinx, and the other is placed in another area of your body such as your abdomen.
• Removing the obstruction. If something within your spinal cord, such as a tumor or a bony growth, is hindering the flow of cerebrospinal fluid, surgically removing the obstruction might restore the flow and allow fluid to drain from the syrinx.
• Correcting the abnormality. If a spinal abnormality is hindering the flow of cerebrospinal fluid, surgery to correct it, such as releasing a tethered spinal cord, might restore fluid flow and allow the syrinx to drain.

Surgery doesn't always restore the flow of cerebrospinal fluid, and the syrinx might remain, despite efforts to drain the fluid from it.

Follow-up care

Syringomyelia can recur after surgery. You'll need regular examinations with your doctor, including periodic MRIs, to assess the outcome of surgery.

The syrinx can grow over time, requiring additional treatment. Even after treatment, some signs and symptoms of syringomyelia can remain, as a syrinx can cause permanent spinal cord and nerve damage.

Lifestyle and home remedies

The following steps might help reduce the effects of syringomyelia.

Avoid activities that can make symptoms worse

Avoid activity that involves heavy lifting, straining or putting force on your spine.

Consider physical therapy

If syringomyelia causes neurological problems that decrease your mobility and activity, such as muscle weakness, pain, fatigue or stiffness, a physical therapist might be able to create an exercise program for you that can help reduce these symptoms.

Talk to your doctor about physical therapists in your area who have expertise in neurological conditions.

Manage chronic pain

If you have chronic pain from your syringomyelia, talk to your doctor about treatment options. Many medical centers have doctors who specialize in pain management.

Coping and support

Living with syringomyelia and its complications can be challenging. Having someone to talk with, whether a friend, counselor or therapist, can be invaluable. Or you might find the support and encouragement you need in a syringomyelia support group.

Ask your doctor to recommend a local group or look for groups online. Support groups provide a forum for sharing experiences and can be good sources of information, offering useful or helpful tips for people with syringomyelia.

Preparing for an appointment

You're likely to start by seeing your family doctor or your doctor might refer you to a doctor trained in brain and nervous system conditions (neurologist).

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as restrict your diet. If you have past medical reports, MRI scans or CT scans that might relate to your problem, bring them to your appointment.

Take a family member or friend to your appointment, if possible, to help you remember the information you'll be given.

Make a list of:

• Your symptoms and when they began
• Key personal information, including spinal or back surgeries or injuries you've had and family history of syringomyelia
• All medications, vitamins or other supplements you take, including doses
• Questions to ask your doctor.

For syringomyelia, questions to ask your doctor include:

• What is likely causing my symptoms or condition?
• Are there other possible causes?
• Is it possible my symptoms will get better on their own?
• What tests do I need?
• What is the best course of action?
• Can exercise help?
• I have other health conditions. How can I best manage them together?
• Are there brochures or other printed material I may have? What websites do you recommend?

What to expect from your doctor

Your doctor is likely to ask you questions, including:

• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?

What you can do in the meantime

Avoid doing anything that worsens your symptoms. For many people with syringomyelia, heavy lifting and straining can trigger symptoms, so avoid these activities. Also, avoid flexing your neck.

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