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Syringomyelia (SM)

Syringomyelia (sih-ringo-my-EE-lee-uh) is a neurological disorder. It is caused when a syrinx--a small cyst or collection of fluid--forms within the spinal cord.(1)

What is Syringomyelia?


Syringomyelia (sih-ringo-my-EE-lee-uh) is a neurological disorder. It is caused when a syrinx--a small cyst or collection of fluid--forms within the spinal cord.(1) Cerebrospinal fluid (CSF) is the watery fluid that surrounds and cushions the brain and spinal cord. When the CSF get blocked, some of it can get into the space where the spinal cord lives. This usually happens when the flow of CSF fluid around the spinal cord or lower brain stem is disrupted by something like scar tissue, tumors, or congenital disorders. Once in the spinal cord space, the CSF collects and expands into the central canal of the spinal column, forming a syrinx. The syrinx can grow and elongate, leading it to pull nerve fibers and cause damage to the spinal cord. Overtime, a syrinx can compress or destroy the nerve tissue.(2) (If the syrinx affects the brain stem, it is called syringobilbia.(3))

 



Image from Mayo Clinic’s website (4) 

 

Risks for and Causes of Syringomyelia


Even though syringomyelia is rare, it is more likely to affect several populations of people5 including the following adults and children:

 

  • Those who have Chiari I and II (the most common cause of congenital syringomyelia)

  • Those who have spina bifida

  • Those with tethered cord syndrome

  • Those with hydrocephalus

  • Those with injuries to spinal cord (sometimes called post-traumatic syringomyelia)

  • Those with spinal cord tumors especially hemangioblastomas and ependymomas

  • Those with inflammation of the arachnoid membrane, also called arachnoiditis (no, not arachnid, like spiders, but related; this is one of the membranes that surrounds and protects the brain and spinal cord. It looks like a spider web, hence the similar name.)

  • Those with meningitis, an inflammation of the meninges, a protective membrane of the brain and spinal cord

 

In some patients, no underlying cause can be found; that's call "idiopathic syringomyelia"--"idiopathic" meaning "we don't know what caused it."

 

Some people have a condition related to syringomyelia called hydromyelia(2) (also called hydrosyringomyelia). In hydromyelia, the central canal running through the center of the spinal cord is abnormally wide. Some medical providers use syringomyelia and hydromyelia interchangeably, though hydromyelia may be present in infants or young child without brain conditions, such as Chiari malformation and spina bifida.

 

Symptoms of Syringomyelia


Some people may have a small syrinx for years, even their whole lifetimes, but never know because it doesn't cause any symptoms. Symptoms usually appear slowly and worsen over time as the syrinx grows bigger. Symptoms also vary based on where the syrinx formed and how large or long it is.

 

Symptoms of syringomyelia include(2):

 

  • Headaches

  • Pain down the arms, in the neck, shoulders into the middle of the back of the legs (radiculopathy); potentially chronic

  • Progressive weakness and muscle atrophy in arms and/or legs

  • Muscles spasms, involuntary twitches (fasciculations) or stiffness in the back, shoulder, necks, arms, and/or legs

  • Loss of reflexes

  • Charcot joints--chronic, progressive degeneration of the joint due to damages to the nerves that supply the joint; seen as swelling and redness of the area

  • Loss of temperature or pain sensitivity, especially in the fingers, hands, arms, and upper chest

  • Loss of feeling spread over the shoulders and back in a cape-like pattern

  • Numbness, burning, tingling, or piercing in arms and/or legs

  • Balance problems and difficulty walking

  • Issues with sexual function

  • Uncoordinated movements (ataxia)

  • Symptoms associated with damage to the autonomic nervous system: loss of bowel and bladder control, excessive sweating, fluctuating blood pressures

  • Horner's syndrome: damage occurs to one of the nerves that supplies the eyes and face, leading to a droopy eyelid, narrowing of the opening between the eyelids, decreased pupil size, and decreased sweating on the affected side of the face

 

Symptoms specific to children:

 

  • Scoliosis (may be the only symptom in children)

  • Toe walking

 

Diagnosis of and Treatments for Syringomyelia


Imaging can help a neurologist see a syrinx if they suspect syringomyelia.(3) Often, a syrinx is found when imaging is being done for another purpose. The usual test is magnetic resonance imaging (MRI). A dynamic MRI can show the flow of the cerebrospinal fluid in the spinal cord and around the syrinx. Lastly, a myelogram with a computed tomography (CT) scan can be used to find a syrinx, tumor, or other spinal issue.

 

If a syrinx isn't causing any symptoms or the symptoms are mild, there may be no treatment other than monitoring the size of the syrinx through future imaging. Non-invasive treatments for syringomyelia include pain management and medications; physical therapy and rehabilitation; and limiting certain activities that cause strain on the spine. Much of the treatment can be symptomatic--treating the symptoms of syringomyelia instead of the cause--if surgery is not needed or not recommended or symptoms are manageable.(3)

 

Syrinxes that are bigger, growing, or causing more aggravating symptoms are usually treated with surgery. Those with Chiari malformation may be encouraged to get posterior fossa decompression, the most common surgery for Chiari malformation, though it should be noted that there is no consensus specific treatment, therapy, or surgery for Chiari malformation.(6) Those with post-traumatic syringomyelia may wish to prevent a syrinx before it is created. This can be done with a surgery called expansive duraplasty which removes scar tissue along the spinal cord.

 

If there is an obstruction, like scar tissue, bone fragments, or tumors, those can be surgically removed. Tumors may need additional treatment, like radiation. If the cause of the syrinx can't be found, draining the syrinx via placement of a small shunt can help it from growing bigger.

 

Though surgery can be successful, there is always the possibility of the syrinx growing back as well as other issues like CSF leaks, infections, and further spinal cord injury. The key to syringomyelia treatment, therapy, and surgery is to create a plan specific to each patient's symptoms, severity, goals, and lifestyle.

 

1.     https://www.ninds.nih.gov/health-information/disorders/syringomyelia#toc-what-is-syringomyelia-

2.     https://rarediseases.org/rare-diseases/syringomyelia/#disease-overview-main

3.     https://my.clevelandclinic.org/health/diseases/6126-syringomyelia

4.     https://www.mayoclinic.org/diseases-conditions/syringomyelia/symptoms-causes/syc-20354771

5.     https://www.ninds.nih.gov/health-information/disorders/syringomyelia#toc-who-is-more-likely-to-get-syringomyelia-

6.     https://rarediseases.org/rare-diseases/syringomyelia/#therapies

 

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