Tuesday, July 24, 2012

Interhemispheric Effective and Functional Cortical Connectivity Signatures of Spina Bifida Are Consistent with Callosal Anomaly.

Malekpour S, Li Z, Cheung BL, Castillo EM, Papanicolaou AC, Kramer LA, Fletcher JM, Van Veen BD. (2012) Interhemispheric Effective and Functional Cortical Connectivity Signatures of Spina Bifida Are Consistent with Callosal Anomaly. Brain Connectivity 2012 Jun 28.
Department of Electrical and Computer Engineering, University of Wisconsin , Madison, Wisconsin.


The impact of the posterior callosal anomalies associated with spina bifida on interhemispheric cortical connectivity is studied using a method for estimating cortical multivariable autoregressive models from scalp magnetoencephalography data. Interhemispheric effective and functional connectivity, measured using conditional Granger causality and coherence, respectively, is determined for the anterior and posterior cortical regions in a population of five spina bifida and five control subjects during a resting eyes-closed state. The estimated connectivity is shown to be consistent over the randomly selected subsets of the data for each subject. The posterior interhemispheric effective and functional connectivity and cortical power are significantly lower in the spina bifida group, a result that is consistent with posterior callosal anomalies. The anterior interhemispheric effective and functional connectivity are elevated in the spina bifida group, a result that may reflect compensatory mechanisms. In contrast, the intrahemispheric effective connectivity is comparable in the two groups. The differences between the spina bifida and control groups are most significant in the θ and α bands.

Factors Associated With Strabismus in Spina Bifida Myelomeningocele.

Anderson HA, Stuebing KK, Buncic R, Mazow M, Fletcher JM. (2012) Factors Associated With Strabismus in Spina Bifida Myelomeningocele. Journal of Pediatric Ophthalmology and Strabismus. 2012 May 8:1-6.



Higher prevalence of strabismus in individuals with spina bifida myelomeningocele (SBM) has previously been attributed to hydrocephalus; however, SBM is associated with many other complications. This study investigates the relation between strabismus and other factors in SBM.


Children aged 3 to 18 years with SBM (n = 112) received an eye examination including assessment of ocular alignment by cover or Hirschberg test. Gestational age, respiratory distress at birth, birth weight, maternal age at birth, number of shunt revisions, and spinal lesion level were also obtained. The relation between these factors and strabismus was analyzed.


Forty-two participants had strabismus. Maternal age (P = .4) and respiratory distress (P = .6) were not significantly related to strabismus. Lower birth weight was suggestive of a relation with strabismus (logistic regression, P = .05) and younger gestational age was related to strabismus (logistic regression, P = .01). Participants who had at least one shunt revision were more likely to have strabismus (Fisher's exact test, P = .038). Spinal lesion level was significantly related to strabismus with increased likelihood of strabismus for spinal lesions closer to the brain (Wald chi-square, 1,100 = 4.29, P = .038).


These findings indicate that several factors are associated with strabismus in SBM. Some of these factors (lower birth weight and younger gestational age) are associated with strabismus in the general population, whereas the association of strabismus and level of spinal lesion may be unique to SBM and may be related to the more severe brain dysmorphology associated with upper level spinal lesions.
Copyright 2012, SLACK Incorporated.
PMID: 22588727

Maternal tea consumption during early pregnancy and the risk of spina bifida.

Yazdy MM, Tinker SC, Mitchell AA, Demmer LA, Werler MM.  (2012) Maternal tea consumption during early pregnancy and the risk of spina bifida. Birth Defects Research. Part A, Clinical and Molecular Teratology. 2012 May 29

Slone Epidemiology Center at Boston University, Boston, Massachusetts. mahsa@bu.edu.


Studies have demonstrated that catechin, an antioxidant found in tea, can reduce the bioavailability of folate. Because periconceptional folic acid intake has been demonstrated to reduce the risk of spina bifida, tea consumption may put pregnant women at risk because of its possible antifolate properties. Using data collected in the Slone Epidemiology Center Birth Defects Study, we examined whether tea consumption during early pregnancy was associated with an increased risk of spina bifida. Mothers of 518 spina bifida cases and 6424 controls were interviewed within 6 months after delivery about pregnancy events and exposures. Data on tea intake were collected during three periods (1976-1988, 1998-2005 and 2009-2010). Logistic regression models were used to calculate odds ratios (ORs) and 95% confidence intervals (CIs), adjusted for study center. Intake of both periconceptional food folate and diet and supplemental folic acid were examined as a potential effect modifier. For 1976 to 1988, ORs were not elevated for daily tea intake. For 1998 and onward, ORs were also close to 1.0, but there was a modest increase for those who drank more than 3 cups/day (OR, 1.92; 95% CI, 0.84-4.38). Among women with total folic acid intake greater than 400 μg, consumption of 3 cups or more of tea per day was associated with an increased risk of spina bifida in 1976 to 1988 (OR, 2.04; 95% CI, 0.69-7.66) and in the later periods (OR, 3.13; 95% CI, 0.87-11.33). Our data do not support an overall association between tea consumption and spina bifida, but there is a suggestion of a possible interaction between higher levels of folic acid intake and tea consumption. Birth Defects Research (Part A) 2012.

The history of the surgical repair of spina bifida.

McClugage SG, Watanabe K, Shoja MM, Loukas M, Tubbs RS, Oakes WJ. (2012) The history of the surgical repair of spina bifida. Child's Nervous System. 2012 Jun 6.

Pediatric Neurosurgery, Children's Hospital, Birmingham, AL, 35233, USA.


INTRODUCTION: The history of surgical spina bifida repair has seen many successes and failures. Many early surgeons attempted treatment of open spina bifida defects before a clear understanding of the disease process or pathology had been discovered.

CONCLUSIONS: The attempts, while often fruitless, served to better our understanding of the disease and represent stepping-stones to the treatment we successfully use today. The present paper reviews the history of the surgical treatment of myelomeningoceles.

Functional disability of children with spina bifida: It's impact on parents' psychological status and family functioning.

Ulus Y, Tander B, Akyol Y, Ulus A, Tander B, Bilgici A, Kuru O, Akbas S. (2012) Functional disability of children with spina bifida: It's impact on parents' psychological status and family functioning. Developmental Neurorehabilitation. 2012 Jun 19.

Department of Physical Medicine and Rehabilitation .


Objective: To evaluate the impact of functional disability of Turkish children with spina bifida (SB) on parents' psychological status and family functioning. Methods: Fifty-four children with SB and parents were included. The Functional Measure for Children (WeeFIM), Beck Depression Inventory (BDI), and Family Assessment Device (FAD) were used. Results: Mothers' BDI scores were significantly higher than fathers' (p < 0.001). No significant effects of the knowledge of having children with SB before birth and the number of children in families on BDI scores and FAD sub-scores were found (p > 0.05). According to multiple regression analysis; significant correlations with fathers' BDI were problem-solving (p = 0.012) and general functioning (p = 0.037) and with mothers' BDI was roles (p = 0.018). Only childrens age was found to be an influential variable on WeeFIM scores (p < 0.001). Conclusion: Spina bifida healthcare should include psychological support to parents of these children and this support should be independent from disability level of children.

Improved Survival Among Children with Spina Bifida in the United States.

Shin M, Kucik JE, Siffel C, Lu C, Shaw GM, Canfield MA, Correa A. (2012) Improved Survival Among Children with Spina Bifida in the United States. Journal of Pediatrics. 2012 Jun 23. 
Division of Birth Defects and Developmental Disabilities, National Center on Birth Defects and, Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA; RTI International, Atlanta, GA.



To evaluate trends in survival among children with spina bifida by race/ethnicity and possible prognostic factors in 10 regions of the United States.


A retrospective cohort study was conducted of 5165 infants with spina bifida born during 1979-2003, identified by 10 birth defects registries in the United States. Survival probabilities and adjusted hazard ratios were estimated for race/ethnicity and other characteristics using the Cox proportional hazard model.


During the study period, the 1-year survival probability among infants with spina bifida showed improvements for whites (from 88% to 96%), blacks (from 79% to 88%), and Hispanics (from 88% to 93%). The impact of race/ethnicity on survival varied by birth weight, which was the strongest predictor of survival through age 8. There was little racial/ethnic variation in survival among children born of very low birth weight. Among children born of low birth weight, the increased risk of mortality to Hispanics was approximately 4-6 times that of whites. The black-white disparity was greatest among children born of normal birth weight. Congenital heart defects did not affect the risk of mortality among very low birth weight children but increased the risk of mortality 4-fold among children born of normal birth weight.


The survival of infants born with spina bifida has improved; however, improvements in survival varied by race/ethnicity, and blacks and Hispanics continued to have poorer survival than whites in the most recent birth cohort from 1998-2002. Further studies are warranted to elucidate possible reasons for the observed differences in survival.

Spatial navigational impairments in hydrocephalus.

Smith AD, Buckley MG. (2012)  Spatial navigational impairments in hydrocephalus. Cognitive Processing. 2012 Jul 18.
School of Psychology, University of Nottingham, University Park, Nottingham, NG7 2RD, UK, alastair.smith@nottingham.ac.uk.


Whilst much is known about the neuropathological consequences of hydrocephalus, there have been comparatively few studies of the cognitive impairments associated with it. Studies using standardised tests of cognitive function have identified a general pattern of impairments, with patients exhibiting particular difficulty on tests of spatial memory and executive function. A strong prediction is that these deficits are likely to affect daily wayfinding behaviour, and we report a study of spatial and navigational abilities in a group of patients with hydrocephalus but without spina bifida. Participants completed a range of experimental tasks assessing spatial cueing behaviour, landmark memory and route-learning, and idiothetic path integration. This patient group was compared to a control sample matched on verbal, spatial, and intelligence measures, and hydrocephalus was found to be associated with relative impairments in each of the tasks. Patients exhibited reduced sensitivity to spatial cueing, less accurate route-learning, poorer memory for landmark objects, and less accurate spatial updating (with particular impairments in the calculation of heading). Overall, these data represent the first empirical demonstration of navigational impairments in hydrocephalus, and we suggest some of the cognitive, neural, and individual differences factors that may contribute to the pattern of performance reported.

Maternal occupational exposure to polycyclic aromatic hydrocarbons and risk of neural tube defect-affected pregnancies.

Langlois PH, Hoyt AT, Lupo PJ, Lawson CC, Waters MA, Desrosiers TA, Shaw GM, Romitti PA, Lammer EJ; and the National Birth Defects Prevention Study.
Maternal occupational exposure to polycyclic aromatic hydrocarbons and risk of neural tube defect-affected pregnancies. 
Birth Defects Res A Clin Mol Teratol. 2012 Jul 17. doi: 10.1002/bdra.23045. 

Texas Center for Birth Defects Research and Prevention, Birth Defects Epidemiology and Surveillance Branch, Texas Department of State Health Services, Austin, Texas. peter.langlois@dshs.state.tx.us.



This study evaluated whether there is an association between maternal occupational exposure to polycyclic aromatic hydrocarbons (PAHs) and neural tube defects (NTDs) in offspring. This is the first such study of which the authors are aware.


Data were analyzed from 1997 to 2002 deliveries in the National Birth Defects Prevention Study, a large population-based case-control study in the United States. Maternal interviews yielded information on jobs held in the month before through 3 months after conception. Three industrial hygienists blinded to case or control status assessed occupational exposure to PAHs. Crude and adjusted odds ratios (ORs) with 95% confidence intervals (CIs) were estimated using unconditional logistic regression.


Of the 520 mothers of children with NTDs, 5.0% were classified as exposed to occupational PAHs, as were 3.5% of the 2989 mothers of controls. The crude OR for PAH exposure was 1.43 (95% CI, 0.92-2.22) for any NTD and 1.71 (95% CI, 1.03-2.83) for spina bifida. Adjusted ORs were smaller in magnitude and not significant. Among women who were normal weight or underweight, the crude OR for spina bifida was 3.13 (95% CI, 1.63-6.03) and adjusted OR was 2.59 (95% CI, 1.32-5.07). Based on estimated cumulative exposure, a statistically significant dose-response trend was observed for spina bifida; however, it was attenuated and no longer significant after adjustment.


Maternal occupational exposure to PAHs may be associated with increased risk of spina bifida in offspring among women who are normal weight or underweight. Other comparisons between PAHs and NTDs were consistent with no association. Birth Defects Research (Part A)