Are some cases of spina bifida combined with cerebral palsy? A study of 28 cases.
(41/232)
AIM: This study is a description of a group of children with spina bifida who present with neurological impairments resembling cerebral palsy. Spina bifida is a complex congenital spinal anomaly causing paraparesis. Some children with spina bifida have neurological impairments, which fit into the definition of cerebral palsy. Extensive spasticity discordant with the level of the spina bifida lesion, upper extremity dysfunction and cognitive impairment are suggestive of concomitant cerebral palsy in these cases. The probable etiology for this problem may be neglected hydrocephalus, meningitis or other brain lesions common in spina bifida. METHODS: In this study we have reviewed our cohort of 365 patients and found 28 cases with the above-mentioned findings. Main evaluation parameters used were the Ashworth scale for spasticity, Green and Banks modified classification for hand function, cognitive function, mental status, ambulation and lesion level. RESULTS: Twenty-eight out of 365 children with spina bifida had neurological impairments resembling cerebral palsy. Their mean age was 59.9+/-41.3 (range, 16.8-31.2) months. Seventy percent of the patients were nonambulatory and therapeutic ambulation only was present in 30% of patients. Seventy percent of the children had spasticity of Ashworth grade 2 or higher. Upper extremity dysfunction, and cognitive impairment were also observed in 80% of the patients involved in this study. The spinal lesion was 60% thoracal and 20% upper lumbar, and none of the cases had signs of spinal tethering. CONCLUSIONS: We believe that this group of children with spina bifida may be regarded as having concomitant cerebral palsy. This fact implies that the management of this group of patients having mixed findings must be changed accordingly. (+info)
Promotor genotype of the platelet-derived growth factor receptor-alpha gene shows population stratification but not association with spina bifida meningomyelocele.
(42/232)
Neural tube defects (NTDs) constitute a major group of congenital malformations with an overall incidence of approximately 1-2 in 1,000 live births in the United States. Hispanic Americans have a 2.5 times higher risk than the Caucasian population. Spina bifida meningomyelocele (SBMM) is a major clinical presentation of NTDs resulting from lack of closure of the spinal cord caudal to the head. In a previous study of spina bifida (SB) patients of European Caucasian descent, it was suggested that specific haplotypes of the platelet-derived growth factor receptor-alpha (PDGFRA) gene P1 promoter strongly affected the rate of NTD genesis. In our study, we evaluated the association of PDGFRA P1 in a group of 407 parent-child triads (167 Caucasian, 240 Hispanics) and 164 unrelated controls (89 Caucasian, 75 Hispanic). To fully evaluate the association of PDGFRA P1, we performed both transmission-disequilibrium test (TDT) and association analyses to test the hypotheses that PDGFRA P1 was (1) transmitted preferentially in SBMM affected children and (2) associated with the condition of SBMM comparing affected children to unaffected controls. We did find that there was a different allelic and genotypic distribution of PDGFRA P1 when comparing Hispanics and Caucasians. However, neither ethnic group showed strong association between SBMM and the PDGFRA P1 region. These findings suggest that PDGFRA P1 does not have a major role in the development of SBMM. (+info)
Bladder pressure and kidney function in children with myelomeningocoele: review article.
(43/232)
Therapy of children with myelomeningocoele should achieve a double goal: on the one hand, obtain continence and, on the other hand, preserve kidney function without complications. The first goal is a social and psychological one, the latter is a vital one. (+info)
Septic ischial bursitis in patients with spinal cord injury.
(44/232)
Septic ischial bursitis is described in 4 patients with spinal cord injury. In these patients a pre-existing ischial bursitis probably became secondarily infected. Because these patients lack sensation, diagnosis may be difficult. The disease process in one patient with a prolonged fever was only recognized after a leucocyte scan detected an abscess extending to the thigh. At surgery it was found that the infection extended from the ischial bursa to the upper lateral thigh. Infection in these patients was due to beta hemolytic streptococcus, S. aureus, and S. epidermidis. The patients all responded well to local drainage and excision of the bursa. (+info)
Polyethylene glycol 4000 vs. lactulose for the treatment of neurogenic constipation in myelomeningocele children: a randomized-controlled clinical trial.
(45/232)
AIM: To compare the therapeutic effectiveness and tolerability of low daily doses of polyethylene glycol 4000 vs. lactulose in the treatment of neurogenic constipation in children with myelomeningocele. METHODS: Sixty-seven children with chronic neurogenic constipation were randomized allocated to receive either polyethylene glycol 4000 (0.50 g/kg) or lactulose (1.5 g/kg) for 6 months. Patients or their parents reported frequency and modality of evacuation and side effects on a diary card. Primary outcome was bowel frequency > or =3/week, and the second one was side effects at the end of treatment. RESULTS: Complete remission of constipation was reported by a significantly (P < 0.01) higher number of patients treated with polyethylene glycol compared with lactulose. At the end of the study, 46% patients of polyethylene glycol group and 22% of the lactulose group were asymptomatic. Compared with lactulose, patients treated with polyethylene glycol reported higher bowel frequency (5.1 vs. 2.9 bowel movements/week, P < 0.01) and reduction of encopresis. Neither lactulose nor polyethylene glycol caused clinically-significant serious side effects and palatability was similar. CONCLUSIONS: Polyethylene glycol 4000 compared with lactulose provided a higher success rate, without significant side effects, for the treatment of constipation in myelomeningocele children. (+info)
Work participation among persons with traumatic spinal cord injury and meningomyelocele1.
(46/232)
OBJECTIVE: To study injury-related and individual factors as predictors of work participation in persons with traumatic and congenital spinal cord injury. DESIGN: Cross-sectional questionnaire study. SUBJECTS: One hundred and eighty-two persons with traumatic spinal cord injury treated in the Spinal Injuries Unit in Sahlgrenska University Hospital, Goteborg, Sweden, and 48 persons with meningomyelocele admitted to the Young Adult Teams in Goteborg, Boras and Skovde, Sweden. METHODS: A structured questionnaire was sent by post. Main outcome variable was participation in work. Logistic regression modelling was used to study the associations between the potential predictors and work participation. RESULTS: Employment rates were 47% in the traumatic spinal cord injury group and 38% in the meningomyelocele group. The presence of other somatic or mental disorder, and neuropathic pain decreased work participation among the men with traumatic spinal cord injury. Among persons with meningomyelocele, better ambulatory status and higher educational level increased work participation. In all groups higher independence in daily activities increased the probability of work participation. According to multivariable modelling carried out for the men with traumatic spinal cord injury, age over 55 years and the presence of mental disorder decreased work participation. CONCLUSION: Our data show that work participation is affected by individual and injury-related factors. Of the latter, many can be affected by rehabilitation. (+info)
Results in the treatment of paralytic calcaneus-valgus feet with the Westin technique.
(47/232)
Between 1988 and 2003, 23 patients with paralytic calcaneus-valgus feet were submitted to the Westin procedure and 17 patients (25 feet) were re-evaluated. Nine patients were male and eight were female. The mean age at the surgical procedure was 8+/-5 years. The aetiology of paralysis was sequelae of poliomyelitis in 6 patients (8 feet) and of myelomeningocele in 11 patients (17 feet). The mean follow-up period was 6+/-6 years. The results were analysed clinically and radiographically considering the decrease of the retropulsion, the patient's satisfaction, and the increase of the lateral tibiocalcaneal angle. Results were considered satisfactory when the patients showed a decrease of the retropulsion during gait, improvement of the gait pattern, and an increase of the tibiocalcaneal angle. As an overall result, 16 patients (94.2%) were satisfied and 1 patient (two feet) dissatisfied with the outcome. The increase of the tibiocalcaneal angle was significant for the myelomeningocele patients (P=0.001), but not for poliomyelitis (P=0.053). No statistical relation between the follow-up period and the increase of the tibiocalcaneal angle was found (r=0.04). The authors concluded that the Westin procedure is a good technique for the treatment of paralytic calcaneus valgus feet with myelomeningocele. (+info)
Structural and functional characterization of bladder smooth muscle in fetal rats with retinoic acid-induced myelomeningocele.
(48/232)
Myelomeningocele (MMC) is the most common cause of neurogenic bladder dysfunction (NBD). We recently developed a novel retinoic acid (RA)-induced MMC model in fetal rats. The objective of this study was to use this model to assess functional and structural characteristics of the detrusor muscle in MMC-associated NBD. Time-dated pregnant Sprague-Dawley rats were gavage fed 60 mg/kg RA dissolved in olive oil or olive oil alone [embryonic day 10 (E10)]. Bladder specimens from olive oil-exposed fetuses (OIL; n = 71), MMC (n = 79), and RA-exposed-no MMC (RA, n = 62) were randomly assigned for functional and histopathological evaluation and protein analysis. Contractility responses to field and agonist-mediated stimulation (KCl and bethanecol) were analyzed. The expression patterns of alpha-smooth muscle actin, myosin, desmin, vimentin, and collagen III and I were analyzed by immunohistochemistry and Western blotting. Spatial and temporal distribution of nerve fibers within the detrusor muscle was monitored by neurotubulin-beta-III throughout gestation. Neither OIL, MMC, nor RA detrusor responded to field stimulation. MMC bladder strips showed a significant decrease in contractility after KCl and bethanechol stimulation compared with OIL and RA bladders. Bladder detrusor morphology and expression patterns of smooth muscle markers were similar between groups. Detrusor muscles in OIL and RA fetuses were densely innervated, possessing abundant intramural ganglia and nerve trunks that branch to supply smooth muscle bundles. In MMC bladders, neurotubulin-beta-III-positive nerve fibers were markedly decreased with advancing gestational age and were almost completely absent at term (E22). We conclude that the biomechanical properties of fetal rat MMC bladders are analogous to that seen in humans with MMC-associated NBD. Decreased nerve density indicates loss of peripheral neural innervation throughout gestation. The early observation of decreased innervation and decreased contractility in the absence of morphologic abnormalities in muscle structure or extracellular matrix supports a pathophysiological hypothesis that denervation is the primary insult preceding the observed alterations in bladder muscle structure and function. (+info)