tethered cord syndrome symptoms

Tethered cord syndrome may develop as a complication of spinal surgery. A fatty tumor on the lower back. By three months of age, the tip of the spinal cord reaches the normal level between T12 and L2 vertebrae. 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. The spinal cord is wrapped in a covering, or sheath, that allows bones and tissues to expand during childhood and adolescence. In some cases, symptoms may be present at birth ( congenital ), while others may not experience symptoms until later in adulthood. 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. Additionally, urologic and anorectal symptoms may be triggered by spinal cord damage. Two boys, 14 and 18 years of age, presented with leg weakness and Research helps us better understand diseases and can lead to advances in diagnosis and treatment. (Photo 3) A … Early treatment can improve outcomes. Adult and pediatric patients vary slightly in their presentation with a higher incidence of pain in the former and incontinence in the latter. Types of spina bifida associated with tethered cord syndrome include an abnormal connection of inelastic tissue to the caudal spinal cord, dermal sinus tract, which extends from the intraspinal connective tissue to the skin (dermal sinus tract), a split spinal cord (diastematomyelia), and a benign fatty mass or tumor (lipoma) continuous to the spinal cord. The filum terminale is a strand of tissue that bridges the spinal cord tip and the tailbone (sacrum). Leg deformities. Associated with tethered cord syndrome, the elongated cord is often noted in children, but less often in adults. This can damage nerves and cause pain. When it is tethered, it pulls during activity, causing pain and other problems. They include 1) bending slightly (over the sink), 2) Buddha sitting with legs crossed (like the Yoga pose) and 3) Baby holding (or equivalent weight) at the waist level. 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). Bassuk AG, Craig D, Jalali A, et al. It has been said that treatment for adult patients with tethered cord syndrome is controversial However, it is clear that in both pediatric and adult patients who have firm evidence of tethered cord syndrome, prompt surgical intervention results in reversal, or at least stabilization, of symptoms in many cases. Questions sent to GARD may be posted here if the information could be helpful to others. Accordingly, the surgical prognosis varies depending upon the presenting symptoms and tethering-producing anomalies. The legs may feel numb or weak, and may lose muscle. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright. If you have problems viewing PDF files, download the latest version of Adobe Reader, For language access assistance, contact the NCATS Public Information Officer, Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 - Toll-free: 1-888-205-2311, expand submenu for Find Diseases By Category, expand submenu for Patients, Families and Friends, expand submenu for Healthcare Professionals. Am J Med Genet A. Affected children may experience involuntary urination or defecation (incontinence) and repeated urinary tract infections. Adult symptoms also include limb muscle atrophy, sensory deficit (numbness), and urinary frequency and urgency accompanied by a sense of incomplete emptying and even incontinence. They include: • Leg and foot weakness and tighter leg and foot muscles • Your child will have many tests when they are Trouble walking or using legs and feet Secondary causes of tethered cord syndrome include tumors, infection or the development of scar tissue (fibrosis) connected to the spinal cord. New York; Thieme, 2010, National Institute of Neurological Disorders and Stoke. 2008;78:869-870. Or re-adhesion or extensive scar formation might follow the surgery. 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. Some have kyphosis (rounded back) and scoliosis (curved spine). Pathophysiology of tethered cord syndrome and similar complex disorders. These resources provide more information about this condition or associated symptoms. In most cases, individuals experience symptoms during childhood. Tethered cord syndrome. If the spinal cord is tethered at its caudal end, and if the spinal cord is unable to grow as fast as the vertebral column in childhood, the spinal cord is stretched beyond its physiological tolerance. If we don't have a program for you now, please continue to check back with us. Many cases with this anomaly leave a portion of the spinal cord protruded through the spinal canal, typically forming a myelomeningocele. Since tethered cord syndrome is a physiological disorder and develops only when it is abnormally stretched, it cannot be connected to genetic factors, unless the congenital susceptibility of spinal cord to oxidative metabolic impairment is proven. It has been reported that this syndrome … In some cases, electromyography (EMG) and nerve conduction studies may be used to assess nerve function. Tethered cord syndrome can also cause difficulties with bladder and bowel control. In babies and children, TCS is nearly always congenital, meaning the child was born with his or her spinal cord attached to the spinal column or to the skin. This makes it hard for the spine to move freely. Discoloration on the lower back. The course of the disorder is progressive. Gupta G, Heary RF, Michaels J. An elastic, extremely extensible filum allows for the ascension of the less elastic spinal cord. of tethered cord syndrome was the recognition that symptoms may be exacerbated by activity. Progressive sensory and motor deficits may affect the legs potentially resulting in numbness, weakness or muscle wasting (atrophy) in the affected areas. There shall be difficulty in the bowel control. Some neurosurgeons may prefer cutting the thickened filum in these cases for the prophylactic purpose. Bladder dysfunction, in particular, is common. Symptoms of Tethered Spinal Cord Syndrome. Neurosurg Focus. http://www.ninds.nih.gov/disorders/tethered_cord/tethered_cord.htm, http://rarediseases.org/rare-diseases/tethered-cord-syndrome/. Do you know of a review article? In 1916, Spiller [15], a neurologist, described two ad-olescent patients who presented with symptoms of tethered cord syndrome that developed subse-quent to strenuous activity. National Institute of Neurological Disorders and Stroke. Get the latest research information from NIH: https://covid19.nih.gov (link is external). Neurosurg Focus. In addition, the spinal cord is continuous to the filum terminale, which is extremely extensible because of its high viscoelasticity. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. Certain activities such as flexing or extending the lower spinal column can put additional tension on the spinal cord and often worsen tethered cord syndrome. There are many spinal disorders that share similar signs and symptoms as tethered cord syndrome (especially in adult onset TCS). Tethered Cord Syndrome. Filippidis AS, Kalani MY, Theodore N, Rekate HL. J Neurosurg 2010;13: 477-483. It is often associated with spina bifida and scoliosis. In order to help clarify the situation, a proposed definition for true tethered cord syndrome limits that this disorder to individuals who exhibit neurological signs and symptoms due to inelastic structures anchoring the caudal end of the spinal cord. A child with tethered spinal cord syndrome will usually develop symptoms. Tethered cord syndrome is a rare neurological condition in which the spinal cord is attached (tethered) to the surrounding tissues of the spine. Results: Patients with occult tethered cord syndrome presents predominantly with urologic symptoms. Common symptoms reported by people with tethered cord syndrome Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome. myelomeningocele, and in some cases of lipomyelomeningocele. In late teenagers and adults, the displacement of the filum located posterior to the cauda equina (a bundle of nerve roots that originate from the lower spinal cord) is a consistent finding. The symptoms of tethered spinal cord can vary depending on the age of your child. In children, surgery to release "untether" the spinal cord is recommended to prevent or reverse progressive neurological symptoms. Common symptoms include Such birth defects, if located in the tail (caudal) end of the spinal cord, can cause tethered cord syndrome. This important feature is proved by the combination of MRI, endoscopy and surgical findings. Factors in adult onset tethered cord syndrome include: They propose that tethering and abnormal tension were already present before the trauma, which worsened the condition. Symptoms of tethered cord syndrome can appear at any time, but mostly happen during your child’s growth spurts. Children with spina bifida, particularly with myelomeningoceles, show a wide variety of symptoms and physical findings depending on the severity of the defect. Children may have several symptoms of tethered spinal cord, including: Some individuals present with tethered cord syndrome at birth (so-called congenital), while others develop the symptomatology in infancy or early childhood. What are the signs and symptoms of TCS? In many individuals, tethered cord syndrome is caused mechanically by an inelastic often-thickened filum terminale. What is the true tethered cord syndrome? If the filum becomes inelastic in an embryo, then the spinal cord tip is anchored and ceases to ascend. After many years of skepticism, tethered cord syndrome is now considered a distinct clinical entity. Tethered spinal cord syndrome. Argawalla PK, Dunn IF, Scott RM, Smith ER. Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. Neurol Res. Neurosurg Focus. Anomalies connected to the caudal end of the spinal cord (true tethered cord syndrome) can be surgically repaired and result in excellent outcome from untethering. It should be warned that slight flexion of the lower (lumbosacral) spine always aggravates back pain by spinal cord stretching. EMG is a test that records electrical activity in skeletal (voluntary) muscles at rest and during muscle contractions. 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. The spinal cord consists of a long bundle of neuronal fibers (axons) and the interneurons that connect sensory and motor fibers within the cord. During CT scanning (a computer tomography) and MRI special techniques are used to create cross-sectional images of vertebrae and nervous system. Please note that NORD provides this information for the benefit of the rare disease community. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site. Comparisons may be useful for a differential diagnosis. February 7, 2006. Any of the following may worsen as … Information on Clinical Trials and Research Studies, COVID-19 Rapid Response Leadership Series, 5 Myths About Orphan Drugs and the Orphan Drug Act. In some cases, symptoms are stabilized in childhood, but become apparent only in adulthood. Yamada S, Won DJ, Pezeshkpour G, et al. back pain, leg pain, weakness in the lower extremities and/or fatigue with walking. Babies may experience delayed motor milestones such as late walking. Have a question? Tethered Spinal Cord Syndrome Information Page. These attachments cause an abnormal stretching of the spinal cord. Symptoms common to adult tethered cord syndrome include constant, often severe back and leg pain, which may extend to the rectum and genital area in some cases. Due to the variation of the growth rate of the spinal cord and the spinal column, the progression of neurological signs and symptoms is highly variable. Tethered cord syndrome can be of a congenital (primary) origin or acquired (secondary or developmental). Progressive sensory and motor deficits may affect the legs potentially resulting in numbness, weakness or muscle wasting (atrophy) in the affected areas. The other fatty anomaly is a lipomyelomeningocele, in which a lipoma extrudes from the spinal canal underneath the lining of the spinal cord (meninges), but covered by normal skin. 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. Tethered cord syndrome may not result in any symptoms early in life, but may become problematic later due to age-related changes in the spine, resulting in: 1.

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