Recurrent bladder infections, urinary or fecal accidents, as well as urinary frequency, hesitancy and urgency may also be signs of TC with or without back and lower extremity symptoms. 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. Children with spina bifida, particularly with myelomeningoceles, show a wide variety of symptoms and physical findings depending on the severity of the defect. 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. You may want to review these resources with a medical professional. The type of surgery varies depending on the mechanical causes, such as an inelastic filum, myelomeningocele, lipomyelomeningocele, and dermal sinus. The severity of the condition and the associated signs and symptoms vary from person to person. Additionally, urologic and anorectal symptoms may be triggered by spinal cord damage. Due to its high viscoelasticity, the filum allows movement of the spinal cord. Babies may experience delayed motor milestones such as late walking. 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. The spinal cord consists of a long bundle of neuronal fibers (axons) and the interneurons that connect sensory and motor fibers within the cord. The legs may feel numb or weak, and may lose muscle. Spina bifida is a birth defect due to incomplete closure of the posterior spinal cord and bony vertebral arch (lamina). (Photo 1) A dimple above the gluteal crease (the crease in the buttocks) (Photo 2) Long hair (longer than 1 inch) growing on the back over the spine. Doctors carefully monitor children who have a tethered cord and no symptoms. 2010;29:E9. The genetics of tethered cord syndrome. This abnormal attachment is associated with progressive stretching and increased tension of the spinal cord as a child ages, potentially resulting in a variety of neurological and other symptoms. Participation in physical activities such as strenuous sports and ballet dancing with high kicks can worsen the signs and symptoms. These resources provide more information about this condition or associated symptoms. Tethered cord syndrome (TCS) after myelomeningocele (MMC) repair (or secondary TCS) is a challenging condition characterized by neurological, orthopedic, and urological symptoms, which are combined with a low-lying position of the conus medullaris and damage to the stretched spinal cord owing to metabolic and vascular derangements. Common symptoms reported by people with tethered cord syndrome In most cases, individuals experience symptoms during childhood. back pain, leg pain, weakness in the lower extremities and/or fatigue with walking. Pathophysiologically, neuronal dysfunction in tethered cord syndrome results partly from inability for the spinal cord neurons to utilize oxygen, that is, the impaired oxidative metabolism, partly due to lack of oxygen supply (ischemic effect), and partly to ion channel dysfunction directly related neuronal membrane stretching. Have a question? Some individuals present with tethered cord syndrome at birth (so-called congenital), while others develop the symptomatology in infancy or early childhood. the child is expected to manifest problems with the bowel and bladder control. 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. Comparisons may be useful for a differential diagnosis. Tethered cord syndrome. Although some authors call these cases acquired, the majority of these cases are mostly developmental, corresponding to the progressive development of excess fibrous connective tissue (fibrosis) in the filum terminale. 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 ⦠Tethered spinal cord syndrome may go undiagnosed until adulthood, when pain, sensory and motor problems, and loss of bowel and bladder control emerge. This makes it hard for the spine to move freely. Tethered cord syndrome is a rare neurological condition. 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. Adult tethered cord syndrome (ATCS) is a rare entity that usually presents with multiple neurological symptoms, including This prevents the spinal cord from moving to keep up with the lengthening of the spine as it grows. Some neurosurgeons may prefer cutting the thickened filum in these cases for the prophylactic purpose. Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. A child with tethered spinal cord syndrome will usually develop symptoms. J Neurosurg 2010;13: 477-483. 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. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. Common symptoms include Yamada S: Tethered cords syndrome in children and adults. Gupta G, Heary RF, Michaels J. Two boys, 14 and 18 years of age, presented with leg weakness and Bassuk AG, Craig D, Jalali A, et al. Neurosurg Clin N Am. 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. rare disease research! Lesions on the lower back. Research helps us better understand diseases and can lead to advances in diagnosis and treatment. It should be warned that slight flexion of the lower (lumbosacral) spine always aggravates back pain by spinal cord stretching. Tethered spinal cord syndrome. 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. Adult and pediatric patients vary slightly in their presentation with a higher incidence of pain in the former and incontinence in the latter. Tethered cord syndrome may develop as a complication of spinal surgery. A diagnosis of tethered cord syndrome is made based upon identification of characteristic signs and symptoms (see the symptom section) that can neurologically locate the lesion to be above the attachment of the anomalies to the spinal cord. Tethered cord syndrome (TCS) is rare, but no one knows exactly how prevalent it is because it commonly goes undiagnosed. Discussion. Available at: http://www.ninds.nih.gov/disorders/tethered_cord/tethered_cord.htm Accessed: 10/13/10. There are many spinal disorders that share similar signs and symptoms as tethered cord syndrome (especially in adult onset TCS). Am Fam Physician. We want to hear from you. 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 Compensatory to the stretching force, the lower (lumbosacral) spinal cord naturally grows more than seen in normal subjects, and becomes elongated. Trauma to the spine results in a band of scar formation attached to the spinal cord and can cause tethered cord syndrome. Or re-adhesion or extensive scar formation might follow the surgery. A conservative ⦠National Institute of Neurological Disorders and Stroke. In adults, symptoms of tethered cord usually develop slowly. 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. Affected children may experience involuntary urination or defecation (incontinence) and repeated urinary tract infections. 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. A fatty tumor on the lower back. Genetic factors are involved in development of anomalous caudal spine and spinal cord, e.g. Yamada S, Won DJ. 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. Tethered cord syndrome affects males and females in equal numbers. For this purpose, a detailed patient history and a thorough clinical evaluation and detailed MRI studies must be carried out. 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. Please note that NORD provides this information for the benefit of the rare disease community. Adult tethered cord syndrome presents with various neurological symptoms such as bladder/bowel disturbance, motor and sensory disturbance, and pain.1â7 A common bowel disturbance is constipation and incontinence, which may recover depending on the severity or duration of the symptoms.2,3 In adult tethered cord syndrome, recovery of bladder ⦠Do you know of a review article? 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). NORD is a registered 501(c)(3) charity organization. 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. 2007;18:531-547. Bladder dysfunction, in particular, is common. It is often associated with spina bifida and scoliosis. The abnormalities in this examination are only shown in patients with an advanced stage of tethered cord syndrome. Deep dimple on the lower back. We remove all identifying information when posting a question to protect your privacy. Symptoms of Tethered Spinal Cord Syndrome. Pathophysiology of tethered cord syndrome and similar complex disorders. It also leads to strain and stretching on the spinal cord. Symptoms in children may be slowly progressive. For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office: For information about clinical trials sponsored by private sources, in the main, contact: Contact for additional information about tethered cord syndrome: 400 N. Pepper Avenue, Colton, CA 92342-1819, Kaiser Permanente Medical Center, Fontana, Contact: Daniel J. After many years of skepticism, tethered cord syndrome is now considered a distinct clinical entity. Patients may merely note an increasing weakness and after prolonged periods of time, muscular atrophy may be recognized. 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. Tethered Spinal Cord Syndrome Information Page. 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. Progressive sensory and motor deficits may affect the legs potentially resulting in numbness, weakness or muscle wasting (atrophy) in the affected areas. of tethered cord syndrome was the recognition that symptoms may be exacerbated by activity. This trend is due to improvement in neurological examinations and in the interpretation of imaging studies. During CT scanning (a computer tomography) and MRI special techniques are used to create cross-sectional images of vertebrae and nervous system. Reversal of longstanding neurological deficits after a late release of tethered spinal cord. Other individuals may not develop any noticeable symptoms until adulthood. In some cases, electromyography (EMG) and nerve conduction studies may be used to assess nerve function. Signs and symptoms of a tethered cord can include the following: A crooked toe. Do you have updated information on this disease? unusual anatomy of the anus. myelomeningocele, and in some cases of lipomyelomeningocele. 2004;26:722-726. Tethered cord syndrome can be of a congenital (primary) origin or acquired (secondary or developmental). Intermittent back and leg with numbness. Filippidis AS, Kalani MY, Theodore N, Rekate HL. 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. Tethered Cord Syndrome. More than 50 percent of the affected individuals experience bladder and bowel dysfunction, manifested by increased frequency or urgency of urination or constipation. Tethered cord syndrome (TCS) is an abnormal attachment of the spine to the tissue around it. National Organization for Rare Disorders (NORD) 55 Kenosia Ave., Danbury CT 06810 • (203)744-0100. Neurosurg Focus. 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. By three months of age, the tip of the spinal cord reaches the normal level between T12 and L2 vertebrae. The most common include: Spine tenderness. Get the latest research information from NIH: https://covid19.nih.gov (link is external). People with Tethered Cord (TC) can have sacral dimples, discoloration, and hairy patches on their lower back that can lead a doctor to investigate further, however, some have no external signs at all. Incomplete bladder emptying is Special physical features such as abnormal curvature of the spine (scoliosis and exaggerated lordosis) are the potential for symptomatic acceleration. If we don't have a program for you now, please continue to check back with us. At surgery, release of arachnoid adhesion must be performed with meticulous technique. The symptoms of tethered spinal cord can vary depending on the age of your child. Argawalla PK, Dunn IF, Scott RM, Smith ER. Neurosurg Focus. In some cases, symptoms are stabilized in childhood, but become apparent only in adulthood. back pain that radiates to the legs, hips, and the genital or rectal areas. 2010;29:E11. 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. They are extradural lesions, intradural-extramedullary lesions, intramedullary lesions extraspinal lesions, and peripheral neuropathy or myelpathy. 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). Early treatment can improve outcomes. The filum terminale is a strand of tissue that bridges the spinal cord tip and the tailbone (sacrum). 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. When it is tethered, it pulls during activity, causing pain and other problems. unusual anatomy of the genitals. The responses to treatment for tethered cord syndrome by repairing myelomeningocele or removal of scarring formation, varies from one person to another. Parents should talk to their physician and medical team about their child's specific problems, associated symptoms and deformity of the spine and spinal cord. An elastic, extremely extensible filum allows for the ascension of the less elastic spinal cord. During gestation, the spinal cord is continuous to the brain and runs in the spinal canal to the tailbone area. Am J Med Genet A. Associated with tethered cord syndrome, the elongated cord is often noted in children, but less often in adults. Features of the condition may include foot and spinal abnormalities; weakness in the legs; loss of sensation (feeling) in the lower limbs; lower back pain; scoliosis; and urinary incontinence. 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 ⦠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. Information on Clinical Trials and Research Studies, COVID-19 Rapid Response Leadership Series, 5 Myths About Orphan Drugs and the Orphan Drug Act. In general, the spinal cord is protected from external insult by two mechanisms; 1) encased in the spinal column, that is, a rigid structure, 2) floating free in the spinal fluid space of the spinal canal. We want to hear from you. Children hit developmental milestones as they grow, whether itâs learning to walk or potty training. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright. Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. It can affect children and adults, but the causes of TCS differ. The result may be nerve damage and severe pain. 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 shall be difficulty in the bowel control. Secondary causes of tethered cord syndrome include tumors, infection or the development of scar tissue (fibrosis) connected to the spinal cord. Copyright ©2021 NORD - National Organization for Rare Disorders, Inc. All rights reserved. (For more information on this disorder, choose “spina bifida” as your search term in the Rare Disease Database.). NORD strives to open new assistance programs as funding allows. Discoloration on the lower back. In the past, the diagnosis of tethered cord syndrome has been controversial and the disorder often still remains unrecognized and underdiagnosed. 2007;23:1-10. Some have kyphosis (rounded back) and scoliosis (curved spine). Symptoms in newborns include: an unusual dimple, raised bump, discoloration or patch of hair on the lower back. Children may have several symptoms of tethered spinal cord, including: For example, in myelomeningoceles and lipomyelomeningoceles that are directly connected to the entire dorsal surface of lumbosacral spinal cord, their neurological deficits are unrelated to spinal cord stretching (tethered cord syndrome). Some researchers, noting that less severe spinal cord traction may remain asymptomatic in childhood, hypothesize that the age of symptom onset is related to the amount of cord stretch. Stetler WR Jr., Park P, Sullivan S. Pathophysiology of adult tethered cord syndrome: review of literature. 2010;29:E1. Hertzler DA, DePowell JJ, Stevenson CB, Mangano FT. Tethered cord syndrome: a review of the literature from embryology to adult presentation. In addition, the spinal cord is continuous to the filum terminale, which is extremely extensible because of its high viscoelasticity. Pathophysiology of tethered cord syndrome and other complex factors. 2010;29:E2. Neurosurg Focus. Normally, the spinal cord ascends in the spinal canal as the spinal column starts to grow faster than the spinal cord at 9th weeks of gestation. 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. 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. However, some researchers believe that trauma alone is not enough to cause the disorder. 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. These attachments cause an abnormal stretching of the spinal cord. Childs Nerv Syst. Won, MD, Associate of Dr. Yamada. 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. In adults, symptoms are aggravated by trauma, maneuvers associated with stretching of the spine (flexion), disc herniation, and spinal stenosis. The exact incidence of the disorder in the general population is unknown. 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. 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. Skeik N, Jabr FI. However, if left untreated, these symptoms may become quite severe. The specific symptoms, severity and progression of tethered cord syndrome vary from one individual to another. 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. This important feature is proved by the combination of MRI, endoscopy and surgical findings. Neurosurg Focus. In individuals with severe arachnoiditis (adhesion of the meninges to the spinal cord) found by MRI or CT scan, careful evaluation of pain and neurological condition is required to find if surgical treatment is warranted. Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. Some others who present with aforementioned complete paraplegia have no neurological benefit from the repair surgery. The in-depth resources contain medical and scientific language that may be hard to understand. Tethered Cord Syndrome Definition. 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. 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). Tethered cord syndrome is a stretch-induced functional disorder associated with the fixation (tethering) effect of inelastic tissue on the caudal spinal cord, limiting its movement. Yamada S, Knierum DS, Mandybur GM, Schultz RL, Yamada BS. Some people may not have any symptoms until they are adults, although this is rare. The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. The course of the disorder is progressive. What is the true tethered cord syndrome? New York; Thieme, 2010, National Institute of Neurological Disorders and Stoke. Factors in adult onset tethered cord syndrome include: What are the signs and symptoms of TCS? In most cases, the abnormal tension of the spinal cord increases over time, but disturbing symptoms often develop quickly during a few weeks. Any of the following may worsen as ⦠(Photo 3) A ⦠Questions sent to GARD may be posted here if the information could be helpful to others. In many individuals, tethered cord syndrome is caused mechanically by an inelastic often-thickened filum terminale. They propose that tethering and abnormal tension were already present before the trauma, which worsened the condition. Symptomatically, patients with these anomalies present with complete or nearly complete paralsysis of lower limbs and total loss of bladder and rectal control. 2007;23:371-375. Because of its functional (physiological) nature, tethered cord syndrome can be reversible if surgically treated in its early stage. 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. In addition, demonstration of spina bifida (bony defect of the lamina) supports a diagnosis of tethered cord syndrome. Tethered cord syndrome refers to a group of neurological disorders that relate to malform Tethered spinal cord syndrome may go undiagnosed until adulthood, when pain, sensory and motor problems, and loss of bowel and bladder control emerge. Consequently, the spinal cord is pulled upwards due to this growth difference. The spinal cord normally moves freely within the spinal column. 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. Results: Patients with occult tethered cord syndrome presents predominantly with urologic symptoms.
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