1/175. eating disorders in adolescents and young women with spina bifida.This is the first report of the association of spina bifida and eating disorders. Five patients were diagnosed rather late in the course of their illness. They all had been overweight premorbidly and had been urged to lose weight for years in order to improve their mobility. As they dieted, they experienced their weight loss as a source of power that could somehow compensate for their losses and neurologic limitations. They responded to a multidisciplinary intervention. Clinicians taking care of patients with spina bifida need to be cognizant that they may be at risk of developing an eating disorder. Such awareness should influence the quality of nutritional counseling (prevention aspect) and the clinical assessment of sudden weight loss (early intervention).- - - - - - - - - - ranking = 1keywords = spina bifida, bifida, spina (Clic here for more details about this article) |
2/175. Neuropathic arthropathy: a forgotten diagnosis? Two recent cases involving the hip.Two cases of neuropathic arthropathy of the hip are reported. One was the first manifestation of tabes dorsalis in a 74-year-old man, whereas the second occurred in a 47-year-old woman with a history of spina bifida and L2-L5 epidural lipoma. Radiographic joint destruction occurred within five and three months, respectively. The main clinical and radiological features of neuropathic arthropathy are reviewed, and diagnostic pitfalls are discussed. There are no specific laboratory tests or histologic findings. This now rare condition should be routinely considered in patients with severe joint destruction contrasting with minimal pain. The reasons for the decision to use trochanteric-iliac coaptation in one of our patients and a wait-and-see approach in the other are explained. The literature is reviewed. Ten cases treated by joint replacement have been reported. However, neither joint replacement nor arthrodesis seem capable of restoring satisfactory hip function.- - - - - - - - - - ranking = 0.16666666666667keywords = spina bifida, bifida, spina (Clic here for more details about this article) |
3/175. Extrarenal nephroblastic proliferation in spinal dysraphism. A report of 4 cases.Four cases of extrarenal nephrogenic proliferation in the sacrococcygeal region with spinal dysraphism are presented. In two of the cases, features of Wilm's or incipient Wilm's tumor were present. The previous literature on sacrococcygeal nephrogenic tissue is reviewed, and the impact of these findings on the histogenesis of extrarenal sacrococcygeal Wilm's tumor is discussed.- - - - - - - - - - ranking = 0.070670188720807keywords = spina, spinal (Clic here for more details about this article) |
4/175. Myelocystocele-cloacal exstrophy in a pedigree with a mitochondrial 12S rRNA mutation, aminoglycoside-induced deafness, pigmentary disturbances, and spinal anomalies.A large Filipino-American family with progressive matrilineal hearing loss, premature graying, depigmented patches, and digital anomalies was ascertained through a survey of a spina bifida clinic for neural crest disorders. deafness followed a matrilineal pattern of inheritance and was associated with the A1555G mutation in the 12S rRNA gene (MTRNR1) in affected individuals as well as unaffected maternal relatives. Several other malformations were found in carriers of the mutation. The proband had a myelocystocele, Arnold-Chiari type I malformation, cloacal exstrophy, and severe early-onset hearing loss. Several family members had premature graying, white forelock, congenital leukoderma with or without telecanthus, somewhat suggestive of a waardenburg syndrome variant. In addition to the patient with myelocystocele, two individuals had scoliosis and one had segmentation defects of spinal vertebrae. The syndromic characteristics reported here are novel for the mitochondrial A1555G substitution, and may result from dysfunction of mitochondrial genes during early development. However, the mitochondrial A1555G mutation is only rarely associated with neural tube defects as it was not found in a screen of 218 additional individuals with spina bifida, four of whom had congenital hearing loss.- - - - - - - - - - ranking = 0.40400352205414keywords = spina bifida, bifida, spina, spinal (Clic here for more details about this article) |
5/175. Bladder cancer arising in a spina bifida patient.We report the case of a 52-year-old patient with spina bifida, neurologic bladder, and a history of recurrent urinary tract infections (UTIs) in whom a bladder cancer was incidentally discovered. Cytology, cystoscopy, and cystography showed nonspecific, extensive inflammatory lesions. Cystography demonstrated a complex of diverticulae and cellules. Pathologic examination of a diverticulectomy specimen revealed a grade III pT3b transitional and squamous cell carcinoma. Because of the similar disease causation (recurrent UTIs, stones, and indwelling catheterization), we suggest extension of the guidelines proposed for patients with spinal cord injuries (ie, annual serial bladder biopsies) to patients with nontraumatic neurogenic bladder.- - - - - - - - - - ranking = 0.84746737107749keywords = spina bifida, bifida, spina, spinal (Clic here for more details about this article) |
6/175. Intradural spinal lipoma of the conus medullaris without spinal dysraphism.A 42-year-old man suffering from progressive left radicular sensory motor loss (L4 level) underwent neurosurgical repair. neuroimaging (RMI) had led to the diagnosis of schwannoma of the filum terminale with lipomatous component. Histological examination visualized a true mature lipoma associated with numerous bundles of more or less dystrophic nerve fibers. This histological benign tumor raised the problem of the genesis of intradural lipomas of spinal cord.- - - - - - - - - - ranking = 0.12720633969745keywords = spina, spinal (Clic here for more details about this article) |
7/175. Terminal Myelocystocele:an unusual presentation.Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord herniated posteriorly through a skin-covered lumbosacral spina bifida. An arachnoid-lined meningocele, continuous with the spinal subarachnoid space, is traversed by the hydromyelic cord. Clinically, this presents with a skin-covered lumbosacral mass, but often no neurological deficit is present. We present a case of terminal myelocystocele in a child born without deficit and without an obvious back mass. diagnosis was delayed until sphincter disturbance and lower limb inequalities developed. We discuss the presentation, imaging and operative findings in this case.- - - - - - - - - - ranking = 0.19493474215499keywords = spina bifida, bifida, spina, spinal (Clic here for more details about this article) |
8/175. Primary lumbosacral Wilms tumour associated with occult spinal dysraphism.A 4-year-old child presenting with sudden-onset paraplegia and a sacral tumour in association with spina bifida occulta is reported. There were no stigmata of spinal dysraphism at birth. Imaging studies confirmed a sacral tumour with extradural extension up to T10 and spinal dysraphism. The histological features of the extradural and sacral components of the tumour were consistent with a Wilms tumour. The differential diagnosis included a primary sacral teratoma containing Wilms tumour elements or a primary extrarenal Wilms tumour arising in association with a spinal dysraphism. There was no clinical response to chemotherapy or radiotherapy.- - - - - - - - - - ranking = 0.3612563352534keywords = spina bifida, bifida, spina bifida occulta, bifida occulta, spina, occulta, spinal (Clic here for more details about this article) |
9/175. Predictors of parental distress after congenital disabilities.This study investigated the relationship between child functional independence, family dynamics, psychosocial factors, and level of distress in parents of children with congenital disabilities. The Vineland Adaptive behavior Scales, the family Assessment Device (FAD), and the Brief Symptom Inventory (BSI) were administered to 60 parents of children with cerebral palsy, 34 parents of children with spina bifida, and 27 parents of children with limb deficiencies. Stepwise multiple regression analyses indicated that the Roles scale from the FAD and the presence of a significant new psychosocial stressor within the previous 6 months were statistically significant predictors of the level of parental distress as assessed by the BSI. Exploration of the extent to which families have adaptive distributions of responsibilities for dealing with domestic tasks, as well as with unrelated life stressors, may be helpful in identifying those parents of children with congenital disabilities who are at risk for distress.- - - - - - - - - - ranking = 0.16666666666667keywords = spina bifida, bifida, spina (Clic here for more details about this article) |
10/175. Split cord malformation with diastematomyelia presenting as neurogenic claudication in an adult: a case report.STUDY DESIGN: This is a report of a rare presentation of a split cord malformation with diastometamyelia. OBJECTIVES: This report draws attention to the fact that the only manifestation of diastmetamyelia in the adult patient may be neurogenic claudication. SUMMARY OF BACKGROUND DATA: patients with split cord malformations and diastometamyelia rarely have symptomatic onset in adulthood. When present, a traumatic event leading to an acute neurologic change is the usual presentation. methods: An adult patient presented with symptoms of neurogenic claudication in the left leg. magnetic resonance imaging examination showed a split cord malformation and diastometamylia at L3-L4 with spinal stenosis of the left hemicord. Decompressive laminectomy and subtotal resection of the bony spur were performed. RESULTS: Two years after decompression, the patient has complete resolution of his leg symptoms and is back to work. CONCLUSIONS: Neurogenic claudication without any objective neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may be the only presentation in the adult with diastometamyelia. decompression to relieve both clinical and radiologic evidence of spinal stenosis obtained excellent outcome.- - - - - - - - - - ranking = 0.042402113232484keywords = spina, spinal (Clic here for more details about this article) |
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