Health risk behaviors among young adults with spina bifida

Aim Persons with spina bifida who adopt unhealthy lifestyles could be at increased risk of adverse health outcomes because the presence of spina bifida may magnify this risk. We estimated overall and age‐specific prevalence of selected health risk behaviors (HRBs) in young people with spina bifida and examined the association between HRBs and depression. Method We performed analyses on data obtained from individuals with spina bifida (n=130; mean age 23y SD 4y 5mo; 64 males, 66 females; 64% lumbosacral lesion; 77% with shunt) who participated in a population‐based survey conducted by the Arkansas Spinal Cord Commission in 2005. Results Compared with national estimates, young people with spina bifida tend to eat less healthy diets, do less exercise, and engage in more sedentary activities. Respondents were less likely to use substances (alcohol, tobacco, illegal drugs), which peaked among 25 to 31 year olds. About 90% saw a doctor in the previous year. Nearly one half reported mild or major depressive symptoms. In the logistic regression analysis after controlling for potential confounders (age, sex, ethnic group, education, employment, marital status, living arrangement, level of lesion, presence of shunt, mobility, self‐rated health and healthcare utilization), major depressive symptoms were associated with current alcohol drinking (adjusted odds ratio: 4.74; 95% CI 1.18–19.04). Interpretation Young adults with spina bifida exhibit unhealthy behaviors that continue into their late 20s. The findings highlight the need to increase awareness of their health risk profiles in the spina bifida community and show opportunities for mental health and health risk screening and counseling by healthcare providers.     

Compound muscle action potentials in newborn infants with spina bifida

The aim of this study was to investigate the relationship between compound muscle action potentials (CMAPs) and neurological impairment in newborn infants with spina bifida. Thirty-one newborn infants (17 males, 14 females, mean gestational age 39 wks [SD 2]; mean birthweight 3336 g [SD 496]) with spina bifida were investigated at a median age of 2 days (range 1-18 d). Motor and sensory impairment and muscle stretch reflexes were assessed and neuroimaging was performed. CMAPs were recorded from the tibialis anterior muscle and the gastrocnemius muscle after percutaneous electrical nerve stimulation. CMAPs were obtained in almost all infants. The area under the curve of the CMAP (CMAP-area) was associated with motor and sensory impairment and with the presence of muscle stretch reflexes, but not with the morphological level of the spinal anomaly. These associations were stronger for the gastrocnemius muscle than for the tibialis anterior muscle. In conclusion, the CMAP-area correlates with neurological impairment in neonatal spina bifida and provides an estimate of residual motor neuron function in affected spinal segments. The assessment of CMAPs after percutaneous electrical nerve stimulation is recommended as an additional instrument to the clinical neurological examination and imaging studies.     

Embryogenesis Why do we need a new explanation for the emergence of spina bifida with lipoma?

Spina bifida with lipoma is a human malformation that most often affects the lumbosacral area. It is a complex morphological type. Its origin is controversial, and none of the previous hypotheses can be retained in view of the most recent advances in experimental embryogenesis. Contrary to earlier opinons, adipocytes cannot arise from meninges, vessels or glial cells and spina bifida cannot be explained by incarceration of mesodermal tissues during primary neurulation or developmental defect at the level of the tail bud. Spina bifida with lipoma, which actually involves all the derivatives of the so-called dorsal mesoderm, must therefore result from abnormal development of this mesoderm, which is induced by the dorsal neural tube. The location of the primary defect (dorsal mesoderm or neural tube) remains to be established. Received: 25 November 1996     

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.     

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.     

Arnold-Chiari-II malformation and cognitive functioning in spina bifida

Spina bifida is a multifaceted neurological condition with complex neuropsychological sequelae. The cognitive outcome in spina bifida has frequently been attributed to the severity of the hydrocephalus. However, because of complex neuropathology, the influence of hydrocephalus alone does not sufficiently explain the deficits in the cognitive profile in spina bifida. To date, little is known of the role of Arnold-Chiari-II malformation (ACM) in the cognitive profile of these patients. Aim of the current study is to delineate the specific contribution of the ACM in spina bifida by comparing children with ACM and those without ACM. 46 children between 6 and 15 years of age underwent a neuropsychological assessment covering intelligence and a wide range of cognitive functions, such as visuo-motor processing, attention, memory, word fluency and speed of information processing. Comparisons were made between patients with ACM (ACM+) and those without ACM (ACM-); all children with ACM+ also had hydrocephalus. Confounding effects of global cognitive impairment were excluded, such that groups were matched on verbal IQ. Because of complex neuropathology, which is inherent to spina bifida, the method applied was based on a comparison of cognitive profiles of the study group with profiles of patients with cerebellar damage and hydrocephalus found in the literature. Impaired visual analysis and synthesis, verbal memory, and verbal fluency, even after correction for global cognitive impairment, were observed in children with ACM. The hypothesis that in addition to impairment in visual analysis and synthesis, which are related to both hydrocephalus and ACM, specific deficiencies in verbal memory and fluency may be attributed to ACM is supported.     

Stem cell-based in utero therapies for spina bifida: implications for neural regeneration

<正>The history:Myelomeningocele–also known as spina bifida–is a devastating congenital anomaly of the central nervous system that is caused by the malformation of the spinal cord and vertebral column during embryogenesis. Depending on the location of the spina bifida lesion on the spine, patients sufferfromneurologicaldysfunctionranging fromparesis and incontinence to complete paralysis. The current standard of care for spina bifida is in utero surgical repair of the defect     

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.     

Cervical spina bifida cystica: MRI differentiation of the subtypes in children

Background Cervical spinal dysraphism is a rare condition with only 37 cases reported in the literature, of which only seven patients had undergone preoperative magnetic resonance imaging (MRI).Objective By using illustrative cases, we demonstrate the value of MRI in distinguishing the two different subtypes of cervical spinal dysraphism, namely, myelocystocele and meningocele.Method Retrospective review of the MRI scans of six patients with cervical (one high thoracic) posterior cystic swellings, which were diagnosed as cervical dysraphism, was done.Results Three patients were diagnosed with myelocystocele and three patients with meningocele. A comparison of MRI features between the two entities, as well as the associated anomalies, is discussed.Conclusion MRI is the imaging modality of choice for suspected cervical dysraphism, prior to surgery. It helps distinguish the subtypes, as well as identify the associated anomalies.     

Disability and quality of life in spina bifida and hydrocephalus

This study examined the impact of severity and type of condition and family resources on quality of life in children with spina bifida and hydrocephalus. A national UK sample of children aged between 6 and 13 years with spina bifida (n=62), hydrocephalus (n=354), and spina bifida plus hydrocephalus (n=128) were identified via the register of the Association for Spina Bifida and Hydrocephalus (ASBAH). Parents completed standardized measures of Child Health Related Quality Of Life (CQOL), family needs survey (FNS), and caregiving self-efficacy scale (CSES) as well as questions on children's health and physical ability. Results showed there were no significant differences in the overall quality of life for the three disability conditions. The overall CQOL was over 1 SD lower for those with spina bifida and hydrocephalus than for children with other physical conditions. Sex and age were not related to overall CQOL. Specific aspects of CQOL differentiated the three groups. Children with spina bifida had poorer CQOL scores on self-care, continence, and mobility/activities whilst those with hydrocephalus had poorer scores on school activities, worries, sight, and communication. Severity of condition and family resources, i.e. CSES and FNS, predicted 32% of the variance in CQOL. Associations were also found between overall CQOL and problems discernible at birth as well as epilepsy. Other factors, including those related to shunts, were not significantly related to CQOL. It was concluded that hydrocephalus is just as great a threat to CQOL as spina bifida. Beyond the general effect of condition severity on CQOL, family resources (as measured by the CSES and FNS) represent an additional influence on CQOL.     

Functional outcome in young adults with spina bifida and hydrocephalus

Introduction Quality of life was studied in 31 adult survivors of spina bifida, each with Verbal or Performance IQ score over 70.Methods Instruments measured physical and occupational function, cognitive/psychological function, somatic sensation, and social interaction in the context of the ability to live independently.Results Domain group means except motor independence were in the average range (±1 standard deviation). Variability within the group with respect to physical phenotype (high spinal lesions were associated with poorer motor independence) and medical history [a greater number of shunt revisions (>4) were associated with poorer functional numeracy] was predictive of quality of life. Both spinal lesion level and number of shunt revisions influenced occupational status.Discussion There was a relation between everyday mathematics, reading skills, and quality of life. The consequences of the physical, medical, and cognitive effects of spina bifida extend into young adulthood and have an impact on quality of life.     

Sexual and reproductive health in young people with spina bifida

This study aimed to identify the sexual and reproductive health knowledge, attitudes, and behaviour of young people, aged between 14 and 23 years, with spina bifida who had attended the Spina Bifida Clinic at a tertiary centre in Melbourne, Australia within the past 10 years. Subjects participated in a structured interview and their parents completed a written questionnaire. Fifty-one (55%) of 93 eligible young people and 69 of 97 parents (71%) participated. Most young people were satisfied with the amount of general sex education they had received. However, 95% stated they had inadequate knowledge about sexual and reproductive health relating to spina bifida and 59% of parents considered they had inadequate knowledge. Thirty-nine percent of young people and 30% of parents had discussed sexuality issues with a doctor. However, 93% of young people and 100% of parents said that they would definitely talk about these issues if the discussions were initiated by their doctor. A significant degree of sexual intimacy was reported, with 60% reporting an intimate relationship, and 25% (10 females, three males) reporting sexual intercourse. Thirty-seven percent of females had experienced unwanted sexual attention, and 30% reported unwanted sexual touching. This study suggests that health professionals should pay greater attention to sexual and reproductive health issues in young people with spina bifida.