Tethered spinal cord syndrome in adults

Twenty-six of 27 patients in whom an ulnar shortening osteotomy had been performed to treat ulnar impaction following distal radius (20 patients) or forearm (6 patients) fractures were evaluated at an average follow-up of 21 months. All but 3 patients were satisfied with the end-result and according to a modified Gartland-Werley score there were 1 excellent, 10 good, 10 fair and 5 poor results. This is distinctly inferior to other reports of mainly non-traumatic indications. Degenerative changes at the distal radioulnar joint were associated with fair and poor results and careful radiological examination of this joint is mandatory before ulnar shortening is performed in posttraumatic ulnar impaction syndrome. Bony union of the osteotomy was achieved at 12 to 16 weeks postoperatively except for 2 cases and there was no difference between transverse (13 cases) and oblique osteotomies (13 cases). We therefore prefer the technically easier transverse osteotomy and recommend the use of 3.5 dynamic compression plates for stabilisation which resulted in a low complication rate in our series and enables early active wrist mobilisation.     

Growth of growth hormone-treated and nontreated children before and after tethered spinal cord release

Children with myelomeningocele (MM) are very short and respond to treatment with recombinant human growth hormone (RHGH) with an acceleration in growth. Following primary closure of the MM, a tethered spinal cord may produce neurologic and orthopedic deformities. We compared the short-term growth rate and length standard deviation score (L-SDS) for stature of 13 prepubertal children with MM (7 males, 6 females, mean age 6.1 +/- 2.5 years) before and after symptomatic tethered spinal cord release (TCR) to an untreated asymptomatic matched control group. We also compared these data to 7 prepubertal children with MM and growth hormone inadequacy who had TCR and were then treated with RHGH. TCR significantly increased the growth rate compared with matched controls (p < 0.01); however, TCR and RHGH in combination provided an increased gain in growth rate and L-SDS over TCR alone (p < 0.01). Tethered spinal cord influences the growth rate of children with MM. Further study is necessary to evaluate the relationship of the tethered spinal cord to growth and treatment of growth failure.     

Urological assessment for tethered cord syndrome of delayed onset following repair of meningomyelocele

PURPOSE AND MATERIALS: Out of 84 patients with meningomyelocele repaired at birth, 14 (15. 7%) children (6 boys and 8 girls, average 8.8 year-old) who underwent cord untethering for tethered cord syndrome of delayed onset (Delayed TCS) were urologically evaluated. RESULTS: Eleven (78.5%) children were detected orthopedically by deterioration of lower extermities function and only 3 (21.5%) were detected urologically. Preoperative urodynamic study, however, revealed in 5 children aggravation of urinary tract dysfunction, including accelerated uninhibited contraction in 4, impaired bladder compliance in 3, decreased bladder capacity in 2 and recurrence of VUR in 1. Although there were no particular urinary symptom for detection of Delayed TCS, acceleration of uninhibited contraction seemed to be one of the most important findings. After untethering, deterioration or normalization of urological dysfunction was not seen. Six of 14 children had favorable clinical results, such as improvement of uninhibited contraction, increasing of bladder compliance and capacity, prolongation of dry time and disappearance of VUR. In 3 children Delayed TCS were detected by postoperative improvement of urinary tract function. Consequently, of 14 children who were evaluated preoperatively and postoperatively 8 (52. 7%) had urological impairment compatible with Delayed TCS. CONCLUSIONS: It is not easy to detect the urological deteriorations associated with Delayed TCS behind initial neuro-urological deficits. Careful and regular follow-up examinations are required to early detection of Delayed TCS. The fact that urinary tract dysfunction improved in some cases after untethering encourages aggressive surgical untehtering of Delayed TCS.     

Man-in-the-barrel syndrome caused by cervical spinal cord infarction

Ichthyosis bullosa of Siemens is a rare autosomal dominant skin disorder whose clinical findings are quite similar to those of epidermolytic hyperkeratosis. The differences between those two diseases include absence of erythroderma and different distributions in the skin in ichthyosis bullosa of Siemens. Recent studies have confirmed that ichthyosis bullosa of Siemens is caused by the mutation in the keratin 2e (K2e) gene, which is expressed in the upper spinous and granular layers. We have identified a sporadic case of ichthyosis bullosa of Siemens; based on diagnosis by histopathological findings, the K2e gene of the patient was analysed. Direct sequencing of PCR products revealed a single base change in sequences encoding the highly conserved end of the 2B rod domain segment of the K2e gene. This mutation results in substitution of the codon for glutamic acid by a codon for lysine in position 493 in K2e (E493K). Mutations of the K2e gene involving five different residue positions (Q187P, T485P, L490P, E493D, E493K and E494K) are known to cause ichthyosis bullosa of Siemens. Of these sites, E493, which is conserved in type I and type II keratin genes, is the most frequently altered amino acid in the K2e gene. These data together suggest that this codon constitutes a hot spot for mutations in the K2e gene.     

Predicting life satisfaction among adults with spinal cord injuries.

Examined life satisfaction among 91 adults with spinal cord injury (SCI) through structured personal interviews that used the Center for Epidemiological Studies Depression Scale, Rosenberg's Self-Esteem Scale, the Life 3 Measure, and a number of items related to satisfaction with various life domains. Results suggest leisure satisfaction was the most significant predictor of life satisfaction, explaining 43% of the variance in the life satisfaction scores; an additional 16% of the variance was explained by self-esteem and health satisfaction. Findings highlight the role of leisure satisfaction in enhancing life satisfaction among individuals with SCI, given the high unemployment rate in this population. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Radiologic and histopathologic findings in a rare case of complex occult spinal dysraphism with association of a lumbar fibrolipoma, neurenteric cyst and tethered cord syndrome

We present the case of a 60-year-old farmer who reported non-specific back pains for the past 30 years. Increasing paralysis of the left leg led to hospitalization. A complex occult spinal dysraphism comprising a fibrolipoma of the conus medullaris, an neurenteric cyst, and a tethered cord syndrome was diagnosed and operated on. The radiological and histological findings are demonstrated.     

Congenital heart diseases in adults

The classification of diabetes into different types is not always straightforward. Mary Burden explains the role of screening procedures in making an effective diagnosis.     

Congenital giant hydronephrosis in adults

Giant hydronephrosis is the term designating the presence of more than 1,000 ml. of fluid in the collecting system. Of 4 cases of congenital hydronephrosis found over a three-year period at the Ochsner Medical Institutions, 2 were associated with unsuspected carcinomas. Hydronephrosis may first be detected as a mass palpable in the abdomen and displacing the intestines on gastrointestinal films. Excretory urography, retrograde pyelography, and angiography confirm the diagnosis. The treatment of choice is nephrectomy.     

Intraoperative monitoring for spinal cord tumor by spinal cord evoked potential following unilateral spinal cord stimulation

We described our experiences with intraoperative spinal cord monitoring in 6 cases of spinal cord tumor. During the operation, spinal cord evoked potential following unilateral spinal cord stimulation was recorded from subdural monitoring electrodes. This series included two cases of intradural extramedullary tumor (one case each of neurinoma and of meningioma) and four cases of intramedullary tumor (2 cases of cavernous angioma, one case each of ependymoma, and of glioblastoma multiforme). Before the removal of the tumor, the spinal cord evoked potential showed lower amplitude or no response on the more affected side in all 6 cases. During the operation, the different intraoperative changes were shown on each side. The authors think that the detection of unilateral damage to the spinal cord is possible in spinal cord evoked potential using unilateral spinal cord stimulation.     

脊髓拴系综合症的手术治疗体会

我院自2005年8月～2009年8月共手术治疗30例脊髓拴系综合症的病人.本文总结我院30例脊髓拴系综合症的手术治疗体会.     

Long-term outcomes and life satisfaction of adults who had pediatric spinal cord injuries

OBJECTIVE: To determine long-term outcomes and life satisfaction of adults who sustained pediatric spinal cord injury (SCI). DESIGN: Structured interview of adults who were 25 years or older who had pediatric SCI. SETTING: Community. PARTICIPANTS: A convenience sample of 46 patients from a total of 81 patients who received care in an SCI program: 1 refused participation, 4 died, and 30 were lost to follow-up. MAIN OUTCOME MEASURES: A structured questionnaire including physical, psychosocial, and medical information. The Craig Handicap Assessment and Reporting Technique and two measures of life satisfaction were also administered. RESULTS: Participants were 25 to 34 years old, mean 27 years. Thirty-two had tetraplegia and 14 had paraplegia. Thirty-one were men. Mean years of education was 14. Fifty-four percent were employed, 48% lived independently, and 15% were married. Life satisfaction was associated with education, income, satisfaction with employment, and social/recreation opportunities, and was inversely associated with some medical complications. Life satisfaction was not significantly associated with level of injury, age at injury, or duration of injury. CONCLUSIONS: Individuals who had pediatric SCI, much like adult-onset SCI, have the greatest opportunity for a satisfying adult life if rehabilitation emphasizes psychosocial factors such as education, employment, and long-term health management.     

Transitory spinal cord swelling in a 6-year-old boy with Guillain-Barré syndrome

Adrenal enlargement was followed by serial ultrasonography in an infant with congenital lipoid adrenal hyperplasia (lipoid CAH) from day 12 until 2 years and 4 months of age, when they were no longer detectable. Contrary to other types of CAH in which the configuration changes soon after replacement therapy, this infant with lipoid CAH showed persistent adrenal cortex enlargement due to massive accumulation of lipids and cholesterol resulting in a damaged glandular cyto-architecture. CONCLUSION: ultrasonographically persistent enlargement of the adrenals after replacement therapy is suggestive of the lipoid form of CAH.     

Cost-effectiveness analysis of spinal cord stimulation in treatment of failed back surgery syndrome

This article presents an analysis of the medical costs of spinal cord stimulation (SCS) therapy in the treatment of patients with failed back surgery syndrome (FBSS). We compared the medical costs of SCS therapy with an alternative regimen of surgeries and other interventions. Externally powered (external) and fully internalized (internal) SCS systems were considered separately. Clinical management models of each of the therapy alternatives were derived from the clinical literature, retrospective data sets, expert opinion, and published diagnostic and therapy protocols. No value was placed on pain relief or improvements in the quality of life that successful SCS therapy can generate. We found that by reducing the demand for medical care by FBSS patients, SCS therapy can lower medical costs. On average, given current screening and efficacy rates, SCS therapy pays for itself within 5.5 years. For those patients for whom SCS therapy is clinically efficacious, the therapy pays for itself within 2.1 years.     

Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release

OBJECTIVE: To investigate the influence of neurosurgical intervention on the appearance of upper motor neuron (UMN) signs in newborns diagnosed with occult spinal dysraphism and tethered cord (TC) during the first month of life. METHODS: A prospective study (1990 to 1996) of 22 consecutive newborns with occult spinal dysraphism monitored for the appearance of UMN signs. Untethering was performed when neurologic or urodynamic investigation indicated the presence of UMN dysfunction. RESULTS: Of 22 patients, 10 remained free of UMN symptoms during follow-up (mean, 67+/-22 months). Untethering was performed in 12 of 22 patients because of the presence of UMN symptoms. In 7 of these 12 patients, there was a documented asymptomatic period of 13+/-11 months before the onset of UMN symptoms. Untethering at a mean age of 18+/-17 months restored normal neurologic and urinary function in all patients (mean postoperative follow-up, 25+/-16 months). Of the 12 children, 5 presented with UMN signs at birth. In these children, untethering was performed at a mean age of 9+/-5 months. In two of these five patients, UMN symptoms did not resolve after surgery, and ongoing conservative bladder treatment was required (mean follow-up, 37+/-14 months). In none of the 12 operated children did signs of retethering occur. CONCLUSIONS: A significant number (10/22) of children born with occult spinal dysraphism and TC did not develop UMN symptoms during follow-up; neurosurgical correction after the appearance of an UMN sign restored normal neurologic and urinary function in all children; and untethering in children presenting at birth with UMN symptoms resulted in poorer outcome.     

The reflex sympathetic dystrophy syndrome in patients who have had a spinal cord injury

Patients suffering from a spinal cord injury often present with a pain syndrome. Although the reflex sympathetic syndrome is a common diagnosis in some forms of neurological disease such as patients with a stroke, it is less frequent in those with a spinal lesion. The authors report eight patients with reflex sympathetic dystrophy who had a spinal cord injury. The diagnosis and treatment are discussed along with a review of literature.     

Ischemic spinal cord disorders

Infarcts in the territory of the anterior spinal artery usually are located in the Adamkiewicz artery. Onset is either sudden or progressive with clinical signs of deficit and spinal pain of short duration. The extent and distribution of the deficit depends on the extension and the level of the spinal lesions. A perfect knowledge of the spinal cord vascularization is required to discern the complex and variable clinical expression. Unlike cerebral vascular events, emboli or cardiac origin are rarely involved in spinal cord events. Aortic pathology, including surgery of the aorta, is however of major importance in ischaemic spinal cord syndromes. Several reports have shown that atheroma causing spinal cord ischaemia are not located in the perispinal arterial network but lie on the aorta and its spinal branches. Dissection of the aorta is found responsible in 2 to 8% of the cases. Isthma stenosis can also give spinal cord signs and syphylitic arteritis is exceptionally encountered. Other rare causes are gas emboli and fibrocalcium emboli.     

The cocoon syndrome: A coping mechanism of spinal cord injured persons.

Institutionalized spinal-cord-injured persons have been noted frequently to spend their bed rest and sleep time with their head covered with bed linens or pillows. The present study determined the prevalence of this behavior and its etiology. Comparison groups of 29 spinal-cord-injured and 80 non-spinal-cord-injured patients at 2 rehabilitation facilities were periodically observed during sleep and bedtimes. Following the observation periods, the Ss who manifested the behavior were interviewed to determine causal factors. Results establish a marked prevalence of the behavior in the spinal-cord-injured population compared to other physical disability types. Interview data suggest that head-covering fulfills a variety of functions related to the coping process, but the reason for its prevalence in the spinal-cord-injured population remains uncertain. (16 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)