Syringomyelia

Syringomyelia is a cyst-like collection of cerebrospinal fluid (CSF) that forms in the spinal cord. Over time, it may damage the spinal cord.

Causes

The fluid-filled cyst is called a syrinx. The spinal fluid buildup may be caused by:

• Birth defects (especially Chiari malformation, in which part of the brain pushes down onto the spinal cord at the base of the skull)
• Spinal cord trauma

  Spinal cord trauma

  Spinal cord trauma is damage to the spinal cord. It may result from direct injury to the cord itself or indirectly from disease of the nearby bones,...

  

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• Tumors of the spinal cord

The fluid-filled cyst usually begins in the neck area. It expands slowly, putting pressure on the spinal cord and slowly causing damage.

Onset of syringomyelia is usually between 25 to 40 years old. Males are more affected than females.

Symptoms

If the condition is due to birth defects, there may be no symptoms until age 30 to 40 years. Symptoms of syringomyelia usually appear slowly and worsen over many years. In the case of trauma, the onset of symptoms may be as early as 2 to 3 months of age. If there are symptoms, they may include:

• Headache

  Headache

  A headache is pain or discomfort in the head, scalp, or neck. Serious causes of headaches are rare. Most people with headaches can feel much better...

  

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• Scoliosis (in children)

  Scoliosis

  Scoliosis is an abnormal curving of the spine. Your spine is your backbone. It runs straight down your back. Everyone's spine naturally curves a b...

  

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• Loss of muscle mass (wasting, atrophy), often in the arms and hands

  Wasting

  Muscle atrophy is the wasting (thinning) or loss of muscle tissue.

  

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• Loss of reflexes in upper limbs
• Increased reflexes in lower limbs
• Spasms or tightness in the leg or hand and arm muscles
• Muscle function loss, loss of ability to use arms or legs

  Muscle function loss

  Muscle function loss is when a muscle does not work or move normally. The medical term for complete loss of muscle function is paralysis.

  

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• Numbness that decreases the feeling of pain or temperature; lowers the ability to feel when the skin is being touched; occurs in the neck, shoulders, upper arms, and trunk in a cape-like pattern; and slowly gets worse over time

  Numbness

  Numbness and tingling are abnormal sensations that can occur anywhere in your body, but they are often felt in your fingers, hands, feet, arms, or le...

  

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• Pain down the arms, neck, or into the middle back or legs
• Weakness (decreased muscle strength) in the arms or legs

  Weakness

  Weakness is reduced strength in one or more muscles.

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• Painless burn or injury of the hand
• Difficulty walking or toe walking in children
• Uncontrollable movements of the eyes (nystagmus)

  Nystagmus

  Nystagmus is a term to describe uncontrollable movements of the eyes that may be:Side to side (horizontal nystagmus)Up and down (vertical nystagmus)R...

  

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• Condition that affects the nerves to the eye and face (Horner syndrome)

  Horner syndrome

  Horner syndrome is a rare condition that affects the nerves to the eyes and face.

  

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• Trouble controlling the bowel or bladder

Exams and Tests

Your health care provider will perform a physical exam and ask about the symptoms, focusing on the nervous system. Tests that may be done include:

• MRI of the head and spine

  MRI of the head

  A head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...

  

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• Spinal CT scan with myelogram (may be done when an MRI is not possible)

  Spinal CT

  A lumbosacral spine CT is a computed tomography scan of the lower spine and surrounding tissues.

  

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Treatment

There is no known effective treatment for syringomyelia. The goals of treatment are to stop the spinal cord damage from getting worse and to improve function.

Medicines and devices can help with pain.

Surgery may be needed to relieve pressure in the spinal cord. Physical and occupational therapy may be needed to improve muscle function and help maximize independence.

Ventriculoperitoneal shunting or syringosubarachnoid shunting may be needed. This is a procedure in which a catheter (thin, flexible tube) is inserted to drain the fluid buildup.

Ventriculoperitoneal shunting

Ventriculoperitoneal shunting is surgery to treat excess cerebrospinal fluid (CSF) in the cavities (ventricles) of the brain (hydrocephalus)....

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Other treatments are available to manage bowel and bladder problems and spasticity.

Outlook (Prognosis)

Without treatment, the disorder may get worse very slowly. Over time, it may cause severe disability.

Surgery usually stops the condition from getting worse. Nervous system function will improve in about 30% of the people who have surgery.

Possible Complications

Without treatment, the condition may lead to:

• Loss of nervous system function
• Permanent disability

Possible complications of surgery include:

• Infection
• Other complications of surgery

When to Contact a Medical Professional

Contact your provider if you have symptoms of syringomyelia.

Prevention

There is no known way to prevent this condition, other than avoiding injuries to the spinal cord. Getting treated right away slows the worsening due to this disorder.