Tethered cord syndrome: a pediatric case study

Tethered cord syndrome, seen in patients with spinal dysraphism, is a progressive neurological deterioration due to stretching of the spinal cord. Spinal dysraphism results from defects during embryonic closure of the neural tube, usually in the lumbosacral region. In tethered cord syndrome an abnormally low conus medullaris is tethered by intradural abnormalities such as a short, thickened filum terminale, fibrous bands, a lipoma or diastematomyelia. This article distinguishes tethered cord syndrome from other forms of occult spinal dysraphism and describes and compares normal anatomy to pathological changes. Clinical symptoms and nursing management of the pediatric patient with tethered cord syndrome will be exemplified by a case study presentation.     

Tethered cord syndrome complicating spina bifida occulta. A case report

The insidious onset of back and/or leg pain, weakness and spasticity of the lower limb, sensory deficits and neurogenic bladder and bowel in a child with spina bifida might represent a tethered cord syndrome. A case report of a child with sudden neurologic deterioration describes this condition. The successful management in children and adults with tethered cord syndrome is improved with early recognition and careful monitoring.     

"Tethered" median nerve stress test in chronic carpal tunnel syndrome

Tension of the median nerve produced by simultaneous extension of the supinated wrist and distal interphalangeal joint of the index finger was noted to result in proximal volar forearm pain radiation in patients with chronic carpal tunnel syndrome. This sign was less frequent in patients with a more acute syndrome. Adhesions between the median nerve and the overlying transverse carpal ligament and the development of a pseudoneuroma can individually or together occur in the chronic carpal tunnel syndrome limiting distal nerve excursion of the tethered nerve during simultaneous wrist and index finger extension.     

Central cord syndrome

Central cord syndrome is a relatively common partial cord syndrome occurring in all age groups and often manifest by a confounding neurological presentation. A level of awareness is necessary to diagnose and manage this syndrome optimally.     

Sch?nlein-Henoch syndrome in adults

Nine adult patients, aged from 16 to 70 years, (mean 42 years), with Sch?nlein-Henoch syndrome were studied during the acute stage of their illness. Each presented with the typical purpuric skin rash, and renal disease was found in every case. Microscopic haematuria and proteinuria were consistently found and red cell casts were seen in urine from five patients. Kidney biopsy performed on nine patients showed focal glomerulonephritis in seven cases and diffuse proliferative nephritis in two. The finding of mesangial deposits of immunoglobulin A (IgA) in seven of eight patients is in agreement with recent reports suggesting that the syndrome is an immune complex disorder. The diffuse proliferative lesion was accompanied by loss of renal function, but there were no fatalities in this series. Corticosteroids administered during the acute stage appear to ameliorate the symptoms, especially the arthralgia and abdominal pain, and may prevent progression of the renal disease in some cases.     

Spinal cord injury in partial Down syndrome

Spinal cord injury is well documented in Down syndrome, and is commonly associated with atlantoaxial instability and ligamentous laxity. A case of partial Down syndrome is presented in which a cervical spine injury resulted in an incomplete quadriplegia. Possible factors leading to increased risk of SCI in partial Down syndrome are ligamentous laxity, atlantoaxial subluxation or odontoid abnormalities, neck inflammatory changes, and age below eight years.     

Secondary tethered cord syndrome in patients with myelomeningocele

Myelomeningocele is associated with other neurological abnormalities, including hydrocephalus, Chiari II malformations, syringomyelia, and secondary tethered cord syndrome. Tethered cord syndrome occurs because of abnormal attachment of the spinal cord to the caudal dural sac, causing cord ischemia. Occasionally, symptoms of progressive neurological deterioration may occur and can significantly affect the long-term outcome of these patients. Proper management of patients with myelomeningocele requires long-term follow-up and evaluation of signs of neurological deterioration that suggest secondary tethered cord syndrome. Treatment of these patients may target symptoms, such as urological intervention for bladder dysfunction, or it may target the tethered cord itself. Recently, many studies have shown that tethered cord release can significantly improve symptoms in these patients.     

Central dysesthesia syndrome in spinal cord injury patients

We have described 13 spinal cord injury patients with a complaint of diffuse, ongoing dysesthesias below the level of the lesion, which are burning in quality, and usually functionally limiting. Quantitative sensory and neurophysiological testing revealed relative preservation of the dorsal column functions in comparison to absence of spinothalamic system mediated functions. On the basis of these findings, we are speculating that such an imbalance between the spinothalamic and dorsal column systems is the main underlying mechanism of dysesthesias as a central nervous system misinterpretation of residual peripheral input.     

脊髓栓系综合征的MRI影像诊断

脊髓栓系综合征(TCS)是一种脊髓先天性发育异常，为脊髓圆锥低位栓系并伴发其他畸形，从而产生一系列神经功能障碍的综合征。传统X线等检查手段常难以确诊，目前诊断主要依靠MRI及CT，尤其是MRI对本病的诊断具有明显优势。     

脊髓栓系综合征MRI表现

目的探讨脊髓栓系综合征的MRI影像表现特征。方法回顾性分析14例脊髓栓系综合征患者的MRI影像资料。结果14例MRI均明确诊断为脊髓栓系综合征;腰椎生理曲度正常7例,生理曲度变浅3例,生理曲度明显加深4例;患者均有脊髓位置下降;终丝横径正常者l例,余13例终丝横径均增粗;12例终丝内可见短T1长T2异常信号,2例终丝信号正常;14例患者中合并脊柱裂2例,双脊髓1例,脊髓空洞2例,骶管囊肿2例,中央管扩张1例,骶管裂1例;脊膜膨出2例,脂肪脊髓脊膜膨出12例。结论MRI对诊断脊髓栓系综合征及手术方案的制定有一定价值。     

Childhood weight and metabolic syndrome in adults.

Metabolic syndrome is a clustering of many insulin resistance-associated cardiovascular risk factors such as hypertension, hypertriglyceridaemia, low high-density lipoprotein (HDL) cholesterol, abnormal glucose metabolism and hyperinsulinaemia. Furthermore, it is known that obesity is the most common clinical state characterized by insulin resistance. Central adiposity, in particular, has been shown to be the most distinctive feature of this syndrome. Some studies have also suggested that obesity per se would be necessary for the expression of metabolic defects associated with centrally distributed fat. It has been presented that undernutrition in utero might 'programme' blood pressure, insulin resistance, blood coagulation and cholesterol metabolism and would thus have a role in the aetiology of cardiovascular disease and type 2 diabetes in adult life. Some studies have also found associations between low birthweight and metabolic syndrome in adulthood. However, criticism on this hypothesis of fetal programming has recently been presented. It has been suggested that the origins of adulthood risk of cardiovascular disease and type 2 diabetes can be related to somatic growth as a child, not necessarily to intrauterine growth. In westernized countries, the relative proportion of underweight newborn children is decreasing, and thus considering entire populations low birthweight has lost its theoretical role in the aetiology of type 2 diabetes and cardiovascular disease. On the other hand, as obesity is known to be increasing in the industrialized countries among all age groups, the association between weight gain in childhood and metabolic syndrome in adulthood is more than noteworthy. Instead of undernutrition during pregnancy, sedentary lifestyle and lack of physical exercise pose a new threat. This results in an increased occurrence of overweight in childhood, which may be the first sign of insulin resistance and future metabolic syndrome.