Syringomyelia


Fact: In most cases, SM it is related to a congenital malformation involving the hindbrain (cerebellum) called Chiari Malformation. SM can also occur as a complication of trauma, meningitis, tumor, arachnoiditis, or a tethered spinal cord

What is Syringomyelia?

Syringomyelia (sear-IN-go-my-EEL-ya) (SM) 
SM occurs when spinal fluid flow builds inside the spinal cord due to blockage from Chiari Mal-formation (CM), which causes a cyst called a Syrinx. It is characterized by the formation of this syrinx in the spinal cord, a fluid-filled space within the substance of the spinal cord. Another description would be that as the volume of spinal fluid increases, it creates a cavity (syrinx) in the spinal cord which results in stretching causing Syringomyelia. Syringomyelia is caused by Chiari Malformation but may also occur as a complication of trauma, meningitis, hemorrhage, a tumor, or arachnoiditis. Over time and if not properly treated, these fluid pockets and the stretching can cause permanent nerve damage or disability.

Different Types of Syringomyelia
In general, there are 2 forms of syringomyelia. Most of the time, the disorder is related to an abnormality of the brain called a, 'Chiari I malformation,' named after one of the doctors who first characterized it. The anatomic abnormality causes the lower part of a person's cerebellum to protrude from its usual location in the back of the head into the cervical or neck portion of their spinal canal. A syrinx might then develop in the cervical region of the person's spinal cord.
Due to the relationship that was once believed to exist between the brain and spinal cord of this form of syringomyelia, doctors at times refer to it as communicating syringomyelia. Symptoms commonly start between the ages of 25-40 and might worsen with straining or any activity that causes CSF pressure to fluctuate suddenly. Some people; however, might experience extended periods of functional stability. Others with this form of the disorder also have hydrocephalus, in which CSF accumulates in the brain, or a condition called, 'arachnoiditis,' in which a covering of the spinal cord is inflamed.
The second major form of syringomyelia happens as a complication of meningitis, trauma, a tumor, hemorrhage, or arachnoiditis. With this form, the syrinx or cyst develops in a segment of a person's spinal cord that has been damaged by one of these conditions. The syrinx then begins to expand. At times this is referred to as, 'non-communicating syringomyelia.' Symptoms might appear months or years after the initial injury beginning with pain, weakness and sensory impairment originating at the site of trauma.
The main symptom of post-traumatic syringomyelia is pain unfortunately, which might spread upward from the site of injury. Symptoms such as numbness, pain, weakness and disruption in temperature sensation can happen on one or both sides of a person's body. Syringomyelia may also adversely affect a person's sweating, sexual function, bladder and bowel control.
Syringomyelia might also involve a part of the brain called the brainstem. The brainstem controls a number of our vital functions such as heartbeat and respiration. When syrinxes affect a person's brainstem, the condition is referred to as, 'syringobulbia.'

Information in this section from:
Disabled World

Common Misdiagnosis

  • Vitamin B12 deficiency: The condition of Vitamin B12 deficiency is a possible misdiagnosis of various conditions, including syringomyelia.
  • Fibromyalgia
  • Chronic Fatigue Syndrome
Symptoms

Symptoms may appear over both short term (months) or long term (years) after the initial injury. Symptoms include:

  • Loss of reflexes
  • Loss of sensitivity to pain 
  • Loss of sensitivity to temperature change
  • Muscle Weakness
  • Sensory Impairment
  • Incontinence (bladder & fecal)
  • Wide Blood Pressure swings
  • Buring in the arms & trunk
  • Decreased feeling in arms, legs & hands
  • Eyelid drooping
  • Pain, midline over the spine

Want to learn more?

The following organizations and/or websites offer additional information and education about Syringomyelia. They are listed in Alphabetical order without any preference or prejudice. Listing these organizations is not a recommendation or referral in any regard for seeking treatment or consultation or support for treatment.


American Association of Neurological Surgeons
American Syringomyelia & Chiari Alliance Project
Canadian Syringomyelia Network
Chiari Connection International
Chiari & Syringomyelia Foundation
Christopher & Dana Reeves Foundation
Christopher S. Burton Syringomyelia Foundation, Inc.
Conquer Chiari
CoRDS Registry
Genetic and Rare Diseases (GARD) Information Center
National Institute of Neurological Disorders And Stroke
National Organization for Rare Diseases
World Arnold Chiari Malformation Association