Syringomyelia is a rare disorder in which a fluid filled cyst forms in the spinal cord. Cerebrospinal fluid surrounds and protects the brain and spinal cord. Syringomyelia happens when this fluid collects within the spinal cord and forms a cyst.
The cyst is called a syrinx. Over time, the syrinx gets bigger and can damage the spinal cord and surrounding nerve fibers.
In this article, learn about the causes and symptoms of syringomyelia, as well as the treatment options and the outlook for a person with this disorder.
In most cases, people have a type of syringomyelia called congenital syringomyelia. An abnormality called a Chiari malformation, which can happen when the fetus is developing in the womb, is responsible for this form of the condition.
In people with a Chiari malformation, the brain tissue extends further than normal from the back of the head into the upper part of the spinal cord. This structural abnormality affects the flow of cerebrospinal fluid and may cause a syrinx to form.
Chiari malformations may happen due to an inherited gene, although researchers are still looking for further evidence of this.
Less commonly, people have a type of syringomyelia called acquired syringomyelia, the causes of which include spinal injuries and other conditions, such as:
Sometimes, there is no known cause of syringomyelia. Doctors call this idiopathic syringomyelia.
Syringomyelia most commonly appears in adults between the ages of 20 and 40 years. Young children and older adults can also develop the condition, however.
Syringomyelia may be slightly more likely to affect males than females.
The symptoms of syringomyelia include:
- muscle weakness in the arms and legs that worsens with time
- pain in the neck and shoulders
- numbness, tingling, or burning sensations
- a decrease in or loss of sensitivity to hot and cold, particularly in the hands
- stiffness and lack of coordination in the legs
- scoliosis, which is a curvature of the spine
- loss of balance
- loss of bladder and bowel control
- difficulties with sexual function
Scoliosis may be the only symptom present in children.
The symptoms of syringomyelia can develop over time, and they usually first appear before the age of 40 years.
Symptoms can sometimes develop suddenly if a person sustains an injury that affects the spinal cord. Coughing or straining can also trigger symptoms in people with existing syringomyelia.
People may experience symptoms on either just one side of the body or both sides.
The main condition that has an association with syringomyelia is a Chiari malformation. There are many different types of Chiari malformation, which is an abnormality in the brain that affects the area where the brain and upper spinal cord join.
Chiari malformations usually happen from birth, although symptoms may not appear until adulthood. People can sometimes get a Chiari malformation later in life due to an injury.
The symptoms of a Chiari malformation vary depending on its severity. Some people have no symptoms, while others may have symptoms that affect their everyday life. In some cases, a Chiari malformation can be life threatening.
Symptoms of Chiari malformations include:
- a headache that spreads upward from the back of the neck
- vision problems
- involuntary eye movements
- muscle weakness
- lack of balance and coordination
Many other neurological conditions can also cause symptoms similar to those of syringomyelia. These conditions include:
- multiple sclerosis
- ankylosing spondylitis
- amyotrophic lateral sclerosis
- central pontine myelinolysis
- spinal muscular atrophy
- diabetic neuropathy
- chronic inflammatory demyelinating polyneuropathy
- arteriovenous malformations
Syringobulbia is a similar neurological condition that also causes a syrinx in the spinal cord. People with syringobulbia have a small slit in the brain stem that affects the cranial nerves and the pathways that control sensory and motor reactions.
To diagnose syringomyelia, a doctor will first take a medical history and ask a person about their symptoms. They will then carry out a variety of tests.
Doctors may use an MRI scan to help them diagnose syringomyelia. This test can also show whether a person has scoliosis.
A moving MRI scan, called a cine or dynamic MRI, shows the movement of cerebrospinal fluid. A doctor can use this to determine whether a syrinx is affecting the flow of this fluid.
Doctors may also use a CT scan to provide a detailed image of the spinal cord and surrounding tissues.
A person may need to undergo motor and sensory tests to assess their movement, balance, and coordination.
Treatment will vary depending on the severity of the symptoms. People may need to work with a team of healthcare professionals to treat syringomyelia.
It may help to limit any activities, such as heavy lifting, that trigger symptoms. Some people may benefit from physical therapy. Pain relief medication can help ease any pain that a person is experiencing.
People with severe or worsening symptoms of syringomyelia may need surgery to create a normal flow of cerebrospinal fluid and drain it from the syrinx. A surgeon can insert a small tube called a shunt into the syrinx to drain the fluid. This drainage should relieve pressure on the spinal cord and ease certain symptoms, including headaches.
If a tumor or tethered spinal cord is causing syringomyelia, people will need specific treatment for this issue.
If a person has syringomyelia but does not experience any symptoms, they will not usually need any treatment. A neurologist will monitor the person's condition in case symptoms start to appear, and they may advise the person to avoid lifting heavy objects or straining the body in other ways.
Surgery for syringomyelia carries a risk of potential complications. Inserting a shunt to relieve pressure on the spine can be effective, but it does have risks.
Shunts can cause injury to the spinal cord and lead to bleeding or infection. A shunt will also need replacing if it develops a blockage or stops working.
Syringomyelia can return after surgery, in which case, a person will need another operation.
If a person with symptoms of syringomyelia does not receive treatment or there is a long delay in treatment, this may result in permanent damage to the spinal cord.
Without treatment, symptoms may worsen. A person may experience:
- chronic pain
- loss of sensation in the hands
- worsening weakness in the arms and legs
In cases where surgery is necessary, it can help improve symptoms or stabilize the condition for most people.
If a person has syringomyelia without any symptoms, a neurologist will monitor them in case any symptoms start to develop, and treatment becomes necessary.