Tethered spinal cord syndrome

Tethered Spinal Cord Syndrome or Occult Spinal Dysraphism Sequence is a rare neurological disorder (occurring in 0.05 to 0.25 of 1000 births) caused by tissue attachments that limit the movement of the spinal cord within the spinal column. These attachments cause an abnormal stretching of the spinal cord. The course of the disorder is progressive. Symptoms may include lesions, hairy patches, dimples, or fatty tumours on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence. Tethered spinal cord syndrome may go undiagnosed until adulthood, when sensory and motor problems and loss of bowel and bladder control emerge. This delayed presentation of symptoms is related to the degree of strain placed on the spinal cord over time. Tethered spinal cord syndrome appears to be the result of improper growth of the neural tube during fetal development, and is closely linked to spina bifida. Tethering may also develop after spinal cord injury and scar tissue can block the flow of fluids around the spinal cord. Fluid pressure may cause cysts to form in the spinal cord, a condition called syringomyelia. This can lead to additional loss of movement or feeling, or the onset of pain or autonomic symptoms.

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Treatment

In children, early surgery is recommended to prevent further neurological deterioration, including but not limited to chronic urinary incontinence. In adults, surgery to free (detether) the spinal cord can reduce the size and further development of cysts in the cord and may restore some function or alleviate other symptoms. Tethered Cord Syndrome is causally linked to Chiari Malformation and any affirmative diagnosis of TCS must be followed by screening for Chiari's several degrees. TCS was just recently related to Ehlers-Danlos Syndrome which should also be screened for upon a positive TCS diagnosis.

Tethered Cord Syndrome presents varied and almost uncontrollable neurophenomenae in pediatric cases.

Other treatment is symptomatic and supportive. NSAIDs, opiates, synthetic opiates, Cox II inhibitors, off-label applications of tricyclic anti-depressants combined with anti-siezure compounds have yet to prove they are of value in treatment of this affliction's pain manifestations. There is anecdotal evidence that TENS units may benefit some patients.

Prognosis

With treatment, individuals with tethered spinal cord syndrome have a normal life expectancy. However, most neurological and motor impairments are irreversible.

References

• Tethered spinal cord syndrome. National Institutes of Health. Retrieved on 2007-11-03.
• Tethered spinal cord syndrome. Neurosurgery Today. Retrieved on 2007-11-03.