Tethered Spinal Cord Syndrome Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column.  Attachments may occur congenitally at the base of the spinal cord (conus medullaris) or they may develop near the site of an injury to the spinal cord.  These attachments cause an abnormal stretching of the spinal cord.  The course of the disorder is progressive.  In children, symptoms may include lesions, hairy patches, dimples, or fatty tumors on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence.  This type of 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.  Tethered spinal cord syndrome may go undiagnosed until adulthood, when pain, 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 and may be exacerbated during sports or pregnancy, or may be due to narrowing of the spinal column (stenosis) with age. 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, feeling or the onset of pain or autonomic symptoms. MRI imaging is often used to evaluate individuals with these symptoms, and can be used to diagnose the location of the tethering, lower than normal position of the conus medullaris, or presence of a tumor or fatty mass (lipoma).  In children, early surgery is recommended to prevent further neurological deterioration. Regular follow-up is important: retethering may occur in some individuals during periods of rapid growth and may be seen between five to nine years of age.  If surgery is not advisable, spinal cord nerve roots may be cut to relieve pain.  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.  Other treatment is symptomatic and supportive. With treatment, individuals with tethered spinal cord syndrome have a normal life expectancy.  However, some neurological and motor impairments may not be fully correctable.  Surgery soon after symptoms emerge appears to improve chances for recovery and can prevent further functional decline. The NINDS conducts and supports research on disorders of the spinal cord.  The goals of this research are to find ways to prevent, treat, and cure these disorders. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&term=spinal+AND+tethered+AND+cord+AND+human[mh]+AND+english[la]&db=PubMed&orig_db=PubMed&filters=on&pmfilter_EDatLimit=5+Years 1 http://clinicaltrials.gov/ct2/results?term= /disorders/tethered_cord/pubs_tethered_cord.htm /disorders/tethered_cord/xml_tethered_cord.htm /unknownpath/xml_tethered_cord.htm V91 National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury CT 06810 orphan@rarediseases.org http://www.rarediseases.org 203-744-0100 Voice Mail 800-999-NORD (6673) 203-798-2291 Federation of voluntary health organizations dedicated to helping people with rare "orphan" diseases and assisting the organizations that serve them. Committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and service.
V213 Spina Bifida Association
4590 MacArthur Blvd. NW
Suite 250 Washington DC 20007-4266 sbaa@sbaa.org http://www.spinabifidaassociation.org 202-944-3285 800-621-3141 202-944-3295 Non-profit association that provides information and referrals through a clearinghouse and toll-free number. Promotes research into the causes, treatment and prevention of Spina Bifida; conducts public awareness campaigns; and encourages socialization and training for people with Spina Bifida.
V221 American Syringomyelia & Chiari Alliance Project (ASAP)
P.O. Box 1586
Longview TX 75606-1586 info@asap.org http://www.asap.org 903-236-7079 800-ASAP-282 (272-7282) 903-757-7456 Non-profit organization that works to improve the lives of people with syringomyelia, Chiari malformations, and related disorders. Publishes a newsletter and offers other written information, videotapes, an annual conference, and other services.