Language development in children with spina bifida

Spina bifida meningomyelocele (SBM) is the most common severely disabling birth defect in North America. It is a disorder of the central nervous system that includes, in addition to the defining spinal dysraphism, congenital malformations of the cerebellum and corpus callosum that, along with hydrocephalus, produces a range of cognitive and motor difficulties, including language. In the language domain, many children with SBM demonstrate adequate development of language at the level of form and content (grammar and lexicon). However, most children with SBM experience significant difficulties in the construction of meaning and in pragmatic communication, both of which require flexible language processing in real time. Assessment and intervention should specifically attend to the development of meaning construction and semantic-pragmatic communication.     

Pain in children and adolescents with spina bifida

The Pediatric Pain Questionnaire and relevant medical records were used to investigate the frequency, intensity, location, and duration of pain experienced by children with spina bifida and to examine the concordance between child self-report and parent proxy report of pain. There were 68 children with spina bifida (30 males, 38 females) between the ages of 8 and 19 years (mean age 12y 8m). Fifty-nine children (87%) were diagnosed with myelomeningocele, six (9%) with lipomyelomeningocele, and three (4%) with lipomeningocele (4%). Forty-nine children (72%) had shunted hydrocephalus. Twenty-six children (38%) ambulated full-time. Fifty-six per cent of children reported experiencing pain once a week or more often. For these children, pain occurred most frequently in the head, back, abdomen, neck, shoulders, legs, and hands. Of the 49 children with shunted hydrocephalus, 43 (88%) reported headaches; 15 of 19 children (79%) without hydrocephalus reported headaches. Children who reported more intense pain also reported more frequent pain and more pain locations. Parents were most reliable at reporting locations of their children's severe pain. Parents of younger children significantly underestimated their children's current pain and worst pain in the past 7 days. Lesion level and ambulatory status were not associated with any of the pain variables. It was concluded that children with spina bifida frequently report clinically significant, yet under-recognized and untreated pain.     

Self-concept in children with spina bifida compared with typically developing children

The literature was systematically reviewed to determine if children with spina bifida have lower self-concept compared with their peers with typical development. Relevant trials were identified by searching electronic databases, supplemented by citation tracking. Of 803 papers initially identified, 15 met the inclusion criteria. Meta-analysis revealed children with spina bifida scored significantly lower than children with typical development for the domains of global self-worth (d=-0.39, 95% confidence interval [CI] -0.65 to -0.12); physical appearance (d=-0.26, 95% CI -0.46 to -0.06); athletic competence (d=-0.45, 95% CI -0.67 to -0.22); social acceptance (d=-0.33, 95% CI -0.55 to -0.11); and scholastic competence (d=-0.43, 95% CI -0.66 to -0.21). There was no difference between the groups for the behavioural conduct domain. Children with spina bifida on average have a lower self-concept than their peers with typical development. Clinicians need to take account of this information in planning the assessment and treatment of this group.     

Age-related differences in executive function among children with spina bifida/hydrocephalus based on parent behavior ratings

Previous research has suggested that adolescents with myelomeningocele and shunted hydrocephalus (MMH) have difficulties with aspects of executive functioning and, in turn, with functional independence. There is little research, however, examining patterns of executive functioning across adolescence in this population. The goal of this cross-sectional study was to examine parent ratings of executive function in children with MMH and in typically developing peers across late childhood and adolescence. Parents of 36 individuals with MMH and 35 typically developing peers, ages 10 to 18 years, completed the Behavior Rating Inventory of Executive Function (BRIEF). The BRIEF is organized into eight scales and two primary indices-Metacognition (MCI) and Behavioral Regulation (BRI). As a whole, the children with MMH had significantly higher BRIEF T-scores, as well as a higher prevalence of clinically significant T-scores across subscales, particularly those representing cognitive control. Effects of group, age, and age-by-group interactions on the mean raw scores of the MCI and BRI were examined using regression analyses. There were significant group effects (p <. 05) for both the BRI and MCI, with the controls having significantly lower mean ratings than the MMH group. There was also a significant contribution of age-by-group interaction on the BRI (p <. 05). Although mean raw scores on the BRI for the MMH group remained stable across ages, mean raw scores in the control group decreased as age increased. Thus, healthy children have age-related improvements in executive control behaviors across adolescence, particularly behavioral control, while children with MMH demonstrate no age-related improvements in parent reported executive behaviors across adolescence. Therefore, children with MMH may continue to require targeted interventions and modifications to address executive dysfunction into young adulthood in order to promote functional independence.     

Urinary zinc and metallothionein in children with spina bifida

Defective embryonic cellular zinc utilization may contribute to abnormal neural tube formation and such a defect may be detectable in children with spina bifida (SB). To investigate this possibility, we examined urinary excretion of zinc and metallothionein (Mt), a cytoplasmic metal-binding protein, in 10 girls and 6 boys (ages 6 months to 19 years) with SB and 16 age-matched control subjects. Mean urinary zinc and Mt concentrations in the SB group were 65% and 72% greater than controls, respectively (p less than 0.05). There was was no evidence of renal dysfunction as judged by urinary creatinine and total protein excretion in the SB children. Increased excretion of zinc and Mt in some children with SB may reflect one or more underlying defects of zinc utilization.     

Risk factors for atherogenesis in children with spina bifida

Introduction Several literature reports have demonstrated that cardiovascular disease is one of the most frequent pathologies in patients with spinal cord injury. Aim The aim of this study is to reveal changes in the lipid profile in non-obese children with spina bifida/myelomeningocele (MMC). Discussion The original results demonstrate the necessity of metabolic surveillance in these patients. Daily physical activity must be ensured early on frequency in non-able-bodied children in order to prevent changes in seric lipoproteins.     

Upper limb cerebellar motor function in children with spina bifida

Purpose  

To investigate upper limb cerebellar motor function in children with spina bifida myelomeningocele (SBM) and in typically developing controls.     

Heterogeneity of muscle fat infiltration in children with spina bifida

Children with spina bifida have well recognized functional deficits of muscle, but little is known about the associated changes in muscle anatomy and composition. This study used water-fat magnetic resonance imaging (MRI) to measure fat infiltration in the lower extremity muscles of 11 children with myelomeningocele, the most severe form of spina bifida. MRI measurements of muscle fat fraction (FF) were compared against manual muscle test (MMT) scores for muscle strength. The FF measurements were objective and reliable with mean inter-rater differences of <2% and intraclass correlation coefficients > 0.98. There was a significant inverse relationship between muscle FF and MMT scores (P ≤ 0.001). Surprisingly, however, muscles with negligible strength (MMT 0–1) exhibited a bimodal distribution of FF with one group having FF > 70% and another group having FF < 20%. The MRI also revealed striking heterogeneity amongst individual muscles in the same muscle group (e.g., 4% fat in one participant's lateral gastrocnemius vs. 88% in her medial gastrocnemius), as well as significant asymmetry in FF in one participant with asymmetric strength and sensation. These results suggest that quantitative water-fat MRI may serve as a biomarker for muscle degeneration which may reveal subclinical changes useful for predicting functional potential and prognosis.     

Interdisciplinary treatment of spina bifida children.

In the Spina Bifida Centre, Niguarda Ca' Granda Hospital (Milan, Italy), from June 1985 to June 1990, 60 spina bifida children have been treated. The results of the rehabilitation programme (including orthopaedic, urological and psychosocial aspects) at the time of the survey were: (a) Neuromotor function: 72% of the patients achieved ambulation, 52% of these being less than 2 years of age, and 20% older than 2 years; 18% were too young for walking. Thus, considering the children's age, about 90% of the subjects achieved the standing position, and 89% achieved ambulation. (b) Bladder function: 83% of the children had a complete urological evaluation; 40% of those with a neuropathic bladder had a hyperreflex type, 54% areflexic, and 6% normoreflexic. Thirty-two percent of the patients had signs of 'high pressure' bladder function. Urinary continence was: 36% > or = 2 hours, 20% < 2 hours, 44% not detectable (age < 2 years). Forty percent of the subjects used intermittent catheterisation. (c) Psychosocial aspect: child adaptation to the disease and to the rehabilitation programme was good in 61% of the patients; family problems were identified in 70% of the patients; enrollment in preschool and school programmes was normal (or with specialist teaching) in about 74%; 33.3% of the subjects had disturbance of affect. The results clearly showed that the interdisciplinary approach favoured the social integration of these children.     

Latex sensitization and allergy in children with spina bifida in Turkey

Purpose  

The latex allergy is known as a very frequent problem of children with spina bifida (SB). The aim of this prospective study is to determine the frequency of latex sensitization and allergy in Turkish children with SB and to evaluate the related risk factors.     

Lumbosacral lipomas with spina bifida

The pathological and metabolic characteristics, as well as the anatomical and functional evolution of lumbosacral lipomas, were studied in a series of 73 patients operated on between 1970 and 1983. The pathological study showed that they are mainly composed of adipocytes, but that they also possess fibrous tissue, vessels, and nerve fibers. Innervated muscle fibers, contracting under proper nerve stimulation, were found in several cases. Lipogenesis and lipolysis are the same in lumbosacral lipomas and in normal fat tissue. However, these lipomas can grow with the rest of the fatty pool. Moreover, spontaneous progressive worsening of the clinical status has been observed in 36% of the cases. Surgery is efficient and not harmful to the patient. Postoperative mortality was nil. Early postoperative worsening occurred in 2.7% of the cases. The 6% rate of late postsurgical deteriorations should be compared to the 36% to 56% rate found when patients are not operated on. Three different mechanisms are responsible for clinical worsening: compression or stretching of the cord, and cord injury on the posterior upper limit of the spinal defect. In each case, one mechanism is prevalent. Three different types of lumbosacral lipomas can thus be individualized. The surgical implications of these data are discussed. The necessity for early and systematic surgical treatment is pointed out.Presented at the 14th Annual Meeting of the Japanese Society of Pediatric Neurosurgery, Kochi 1986     

Covert orienting to exogenous and endogenous cues in children with spina bifida

Children with spina bifida meningomyelocele and hydrocephalus (SBM) have congenital dysmorphology of the midbrain and thinning of the posterior cortex, brain regions associated with the control of covert orienting. We studied cued covert orienting in 92 children with SBM, and 40 age-matched typically developing controls. Cues were of three types: exogenous (luminance change in a peripheral box either valid or invalid for upcoming target location), endogenous arrow (a central arrow either valid or invalid for upcoming target location), or endogenous word (a central word either valid or invalid for upcoming target location). Compared to controls, children with SBM showed slowed covert orienting to both exogenous and endogenous cues and a higher cost of attentional disengagement (e.g., a greater cue-validity effect) for exogenous although not for endogenous cues. Covert orienting deficits were associated with midbrain dysmorphology in the form of beaking of the tectum, and with right posterior brain volume loss.     

Neuroradiological assessment of visuoperceptual disturbance in children with spina bifida and hydrocephalus

The intellectual status of twelve children with spina bifida and hydrocephalus was evaluated. Seven children were considered to have verbal and non-verbal skill discrepancies. It was considered that the Verbal IQ-Performance IQ score by WISC-R could be used as an index of their visuoperceptual disturbance. The disturbance was closely related to the morphological characteristics of the lateral ventricles on cerebral MRI. The ratio of the areas of the posterior horns to the anterior horns (P:A) showed a negative correlation with visuoperceptual ability. The visual pathway, visual cortex, and the ventral system were thus considered to be the sites of the affected lesions. From P.A changes with time, it was evident that adequate shunting would prevent the perceptual disturbance.     

Medical complication in adults with spina bifida

Setting

Cohort study.

Introduction

Spina bifida (SB) is a congenital malformation affecting the central nervous system (CNS) and is one of the most prevalent CNS disorders in children. Hydrocephalus (HC) is present in 80% of newborns with SB. The aim of the present study was to analyze the medical complications and to relate the complications to age at examination, the level of injury, AIS grade and presence of HC in adults with SB.

Materials and methods

SB patients were recruited from the Spinalis out-patient clinic at the Karolinska University Hospital at their annual follow-up. The patients underwent a thorough general and neurological examination and background data including medical complications were retrieved from the medical files.

Results

127 of 157 (82%) SB patients (114 with MMC and 13 SB occulta) with a mean age of 34 years were included. Half of the patients had a complete SCI and a lumbar level was most common. Nearly 60% of the patients had HC. 88 patients (69%) suffered from at least one medical complication. Urinary tract infection (UTI), scoliosis and pain were the most common complications found in 46%, 30% and 28% of the patients, respectively. Less common complications were epilepsy, pressure ulcers (PU) and spasticity.

Discussion

SB gives a disability including motor, sensory dysfunctions and the patients suffer from a high frequency of medical complications like UTI, scoliosis, pain, and epilepsy. Data gives basis for adequate routines for medical examination at the follow-up.     

Prospective memory in adults with spina bifida

Introduction  

Individuals with neurodevelopmental disorders have been observed to show accelerated cognitive aging or even dementia as early as 30 and 40 years of age. Memory deficits are an important component of age-related cognitive loss.     

Intramedullary spinal teratoma with spina bifida

Spinal intramedullary teratoma is a rare tumor. We report a case of intramedullary teratoma of the conus medullaris with spina bifida. A 5-year-old boy was admitted to our hospital for removal of a lumbosacral tumor sited in an intramedullary location at the conus medullaris, which was totally resected with neuroendoscopic assistance. The pathological diagnosis was mature teratoma consisting of three germ cell layers. The pathogenesis of spinal intramedullary teratoma is discussed with special reference to spina bifida. Received: 21 July 1999 Revised: 21 December 1999