tethered cord syndrome symptoms

Affected children may experience involuntary urination or defecation (incontinence) and repeated urinary tract infections. Occult spinal dysraphism sequence; Tethered spinal cord syndrome; Segmental vertebral anomalies; Occult spinal dysraphism sequence; Tethered spinal cord syndrome; Segmental vertebral anomalies; Occult spinal dysraphism, placeholder for the horizontal scroll slider, Office of Rare Disease Research Facebook Page, Office of Rare Disease Research on Twitter, U.S. Department of Health & Human Services, Caring for Your Patient with a Rare Disease, Preguntas Más Frecuentes Sobre Enfermedades Raras, Como Encontrar un Especialista en su Enfermedad, Consejos Para una Condición no Diagnosticada, Consejos Para Obtener Ayuda Financiera Para Una Enfermedad, Preguntas Más Frecuentes Sobre los Trastornos Cromosómicos. The inelastic structures in children originated from defective closure of the neural tube (the precursor of the spinal cord) during embryonic development, eventually forming a condition known as spina bifida. This structure, which is composed of glial tissue (the supportive structure of nerve cells) and covered by pia mater, is a delicate strand of fibrous tissue, bridging the spinal cord tip and the sacrum (the tailbone). 2010;29:E2. NORD is not a medical provider or health care facility and thus can neither diagnose any disease or disorder nor endorse or recommend any specific medical treatments. Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. 2007;23:1-10. In children, surgery to release "untether" the spinal cord is recommended to prevent or reverse progressive neurological symptoms. To circumvent this problem, two special surgical procedures have been advocated: 1) transection of the spinal cord to relieve severe back and leg pain, and 2) shortening of the spinal column by resection of one or two vertebrae to relieve spinal cord tension. 2007;23:371-375. Lesions on the lower back. A child with tethered spinal cord syndrome will usually develop symptoms. There are cases in which individuals have the symptoms and signs similar to true tethered cord syndrome, but have associated defects that cause compression and impaired blood flow of the spinal cord, or congenital neuronal dysgenesis (failure of neuronal development). Neurosurg Focus. Tethered cord syndrome (TCS) is an abnormal attachment of the spine to the tissue around it. Hertzler DA, DePowell JJ, Stevenson CB, Mangano FT. Tethered cord syndrome: a review of the literature from embryology to adult presentation. In children, typical imaging features such as a low lying spinal cord and a thickened filum terminale is confirmed by special imaging techniques such as magnetic resonance imaging (MRI) or computed tomography (CT) scan and ultrasound studies. There are many spinal disorders that share similar signs and symptoms as tethered cord syndrome (especially in adult onset TCS). Children with spina bifida, particularly with myelomeningoceles, show a wide variety of symptoms and physical findings depending on the severity of the defect. Bassuk AG, Craig D, Jalali A, et al. Tethered spinal cord syndrome. If abnormal fibrous tissue grows into the filum and replaces glial tissue, the filum loses its elasticity and abnormally fixes (tethers) the spinal cord, and becomes the mechanical cause of tethered cord syndrome. Adult and pediatric patients vary slightly in their presentation with a higher incidence of pain in the former and incontinence in the latter. The course of the disorder is progressive. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright. To search for patient organizations and other pages related to this topic, use the Advanced Search function at the top right corner of the page. They propose that tethering and abnormal tension were already present before the trauma, which worsened the condition. Progressive sensory and motor deficits may affect the legs potentially resulting in numbness, weakness or muscle wasting (atrophy) in the affected areas. Such birth defects, if located in the tail (caudal) end of the spinal cord, can cause tethered cord syndrome. Copyright ©2021 NORD - National Organization for Rare Disorders, Inc. All rights reserved. Neurosurg Focus. Tethered spinal cord syndrome may go undiagnosed until adulthood, when pain, sensory and motor problems, and loss of bowel and bladder control emerge. Because of its functional (physiological) nature, tethered cord syndrome can be reversible if surgically treated in its early stage. Children may have several symptoms of tethered spinal cord, including: The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional. Genetic factors are involved in development of anomalous caudal spine and spinal cord, e.g. Neurol Res. In surgically treated patients, pain relief can often be achieved, and long-term neurological stabilization tends to persist more often than it does in conservatively treated patients. In an individual with only minimum complaint his/her physician may advise conservative treatment rather than surgery and will monitor the condition to see whether the symptoms progress Many experts of tethered cord syndrome recommend against surgery to individuals who present with the MRI finding of "cord elongation and thickened filum" but have no symptoms. Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered 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. The responses to treatment for tethered cord syndrome by repairing myelomeningocele or removal of scarring formation, varies from one person to another. Pathophysiology of tethered cord syndrome and other complex factors. the child is expected to manifest problems with the bowel and bladder control. Babies may experience delayed motor milestones such as late walking. Results: Patients with occult tethered cord syndrome presents predominantly with urologic symptoms. Symptoms in children may be slowly progressive. Tethered cord syndrome can also cause difficulties with bladder and bowel control. Patients must rely on the personal and individualized medical advice of their qualified health care professionals before seeking any information related to their particular diagnosis, cure or treatment of a condition or disorder. NORD gratefully acknowledges Shokei Yamada, MD, PhD, FACS, Professor and Former Chairman, Department of Neurosurgery, Loma Linda University, for assistance in the preparation of this report. TCS may be present at birth. In adults, symptoms are aggravated by trauma, maneuvers associated with stretching of the spine (flexion), disc herniation, and spinal stenosis. Although genetic factors are found in patients with myelominingocele, more research is necessary to determine the exact role that genetic factors play in the development of stretch-causing anomalies. The exact incidence of the disorder in the general population is unknown. Argawalla PK, Dunn IF, Scott RM, Smith ER. Patients may merely note an increasing weakness and after prolonged periods of time, muscular atrophy may be recognized. When it is tethered, it pulls during activity, causing pain and other problems. In most cases, the abnormal tension of the spinal cord increases over time, but disturbing symptoms often develop quickly during a few weeks. If we don't have a program for you now, please continue to check back with us. When the spinal cord becomes tethered, it’s attached to nearby tissues. The severity of the condition and the associated signs and symptoms vary from person to person. Tethered cord syndrome (TCS) is a condition that can be present in EDS, where the spinal cord is attached to surrounding tissue in a way that creates elongation and tension of the nervous tissue, leading to low back pain, loss of bladder control, lower body weakness, and loss of sensation. Adult tethered cord syndrome (ATCS) is a rare entity that usually presents with multiple neurological symptoms, including It should be warned that slight flexion of the lower (lumbosacral) spine always aggravates back pain by spinal cord stretching. In others, where the anomalous structure is attached to the wide area of the spinal cord, signs and symptoms reflect local effects on the spinal cord, and not stretched-induced dysfunction (tethered cord syndrome). Associated with tethered cord syndrome, the elongated cord is often noted in children, but less often in adults. Participation in physical activities such as strenuous sports and ballet dancing with high kicks can worsen the signs and symptoms. Comparisons may be useful for a differential diagnosis. The type of surgery varies depending on the mechanical causes, such as an inelastic filum, myelomeningocele, lipomyelomeningocele, and dermal sinus. Intermittent back and leg with numbness. Bladder dysfunction, in particular, is common. Or re-adhesion or extensive scar formation might follow the surgery. If the filum becomes inelastic in an embryo, then the spinal cord tip is anchored and ceases to ascend. Causes. Adult onset of tethered cord syndrome was considered to be rare for many years, but an increasing number of cases have been reported in recent years. These resources provide more information about this condition or associated symptoms. Additionally, urologic and anorectal symptoms may be triggered by spinal cord damage. Some researchers have speculated that some cases of tethered cord syndrome that occur due to anomalies that can cause stretching of the spinal cord may have a genetic basis or that some individuals are genetically predisposed to developing the disorder in these specific cases. Contact a GARD Information Specialist. Do you know of a review article? During gestation, the spinal cord is continuous to the brain and runs in the spinal canal to the tailbone area. The most common include: Spine tenderness. Some others who present with aforementioned complete paraplegia have no neurological benefit from the repair surgery. The most important signs that can be found in late teenagers and adults are back pain aggravated immediately on flexion of the lumbosacral spine, which elongates the lumbosacral spinal canal, simultaneously stretching the lower spinal cord. These attachments cause an abnormal stretching of the spinal cord. Tethered cord syndrome is a broadly used term for progressive neurological deterioration localized to lower spinal cord abnormalities (such as fibrous bands or adhesions, thickened filum terminale, diastematomyelia, or intradural lipoma), resulting in traction on the conus medullaris.1,2 The family physician can see patients with tethered cord syndrome …

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