Syringomyelia Causes, Symptoms, Diagnosis, Treatment, Prevention And Home Remedies

Syringomyelia Causes, Symptoms, Diagnosis, Treatment, Prevention And Home Remedies

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. In most cases, the disorder is related to a congenital abnormality of the brain called a Chiari I malformation. This malformation occurs during the development of the fetus and 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. Syringomyelia may occur as a complication of trauma, meningitis, hemorrhage, a tumor, or arachnoiditis. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma. Some cases of syringomyelia are familial, although this is rare.

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.

The following conditions and diseases can lead to syringomyelia:

  • 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
  • Tethered spinal cord syndrome - a disorder caused when tissue attached to your spinal cord limits its movemen
  • Spinal injury - which may cause symptoms months or even years after the initial injury
  • Spinal scar tissue -which can develop after surgery

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.

Symptoms of Syringomyelia

Symptoms of syringomyelia usually develop slowly over time. If your syringomyelia is caused by Chiari malformation - a condition in which brain tissue protrudes into your spinal canal — symptoms may begin during your teenage years or early adulthood. In some cases, a fall, minor trauma, coughing or straining may trigger symptoms of syringomyelia, although none of these causes syringomyelia.

The following early signs and symptoms of syringomyelia may affect the back of your 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 your back, shoulders, arms and legs
  • Pain in your neck, arms and back
  • Bowel and bladder function problems
  • Muscle weakness and spasms in your legs
  • Facial pain or numbness
  • Spinal curvature (scoliosis)

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.

Follow-up care

Follow-up care after surgery is critical because syringomyelia may recur. You'll need regular examinations with your doctor, including periodic MRI tests, to assess the outcome of surgery. Other syrinxes may form, requiring additional surgery. Even after treatment, some signs and symptoms of syringomyelia may remain, as a syrinx can cause permanent spinal cord and nerve damage.

Prevention of Syringomyelia

There is no efficient way to predict who will develop syringomyelia. Still, since it is known that certain injuries and infections may contribute to the condition, by avoiding these one may prevent syringomyelia from developing.

What is more important is prevention of complications syringomyelia itself brings. So, only timely performed surgery may prevent irreversible damage to the spinal cord and life-long neurological sequelae. 

Home Remedies

Avoid activities that may make symptoms worse

If you've been diagnosed with syringomyelia, avoid any activity that involves heavy lifting, straining or putting excessive force on your spine. Examples of activities to avoid include:

  • Playing high-impact sports
  • Riding roller coasters
  • Sky diving
  • Straining during a bowel movement
  • Excessive coughing (talk to your doctor about treatment if coughing persists)

Consider physical therapy

If syringomyelia causes ongoing neurological problems that decrease your mobility and activity — such as muscle weakness, pain, fatigue or stiffness — a physical therapist may 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

Chronic pain can be a problem with syringomyelia. If you're experiencing chronic pain, talk to your doctor about treatment options. Many medical centers have doctors who specialize in pain management. Often, the best approach for treatment of chronic pain due to syringomyelia is to have a health care team that includes your neurosurgeon, neurologist, a rehabilitation specialist and a pain management physician who can work together to create a plan that will best fit your situation.