Cyst impinges on spinal cord

DEAR DR. GOTT: My 16-year-old granddaughter has recently been diagnosed with stage 1 syringomyelia. From what I have read, the prognosis is not good. What can you tell me about this disorder? My granddaughter is a high school junior. She is very intelligent, works hard and is even taking college-prep classes.

DEAR READER: Syringomyelia is a disorder in which a cyst, known as syrinx, forms within the spinal cord. Over time, the cyst enlarges and elongates, destroying the center of the spinal cord in the area where it forms.

In many cases, syringomyelia is also associated with a congenital abnormality known as Chiari I malformation, a condition in which the part of the brain called the cerebellum protrudes into the neck portion of the spinal canal. Trauma, meningitis, tumor, arachnoiditis or hemorrhage may result in the development of a syrinx. In rare instances, the condition is inherited.

Symptoms typically present between the ages 25 and 40, making the fact that your daughter was diagnosed at only 16 worrisome. Symptoms can include pain, weakness and stiffness of the back, shoulders, arms or legs and a loss of the ability to feel extremes of cold or hot, especially in the hands, and headaches. These tend to develop slowly but may occur suddenly because of coughing or straining.

If no symptoms are present, treatment is unnecessary, but the situation should be monitored closely so that should they appear, early treatment can be given.

Surgery is typically the recommended course of action, but, unfortunately, it is not a sure cure. They are, however, the best option for reducing the risk of permanent spinal damage and for stabilizing symptoms.

People generally have worsening symptoms when straining or performing activities that cause cerebrospinal fluid pressure to fluctuate. Some patients may experience long periods of stability in which symptoms are minimal or absent.

You can learn more at the National Institute of Neurological Disorders and Stroke’s syringomeylia information page at www.ninds.nih.gov/ disorders/syringomyelia.

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