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Linda W. Xu, Silvia D. Vaca, Joy Q. He, Juliet Nalwanga, Christine Muhumuza, Joel Kiryabwire, Hussein Ssenyonjo, John Mukasa, Michael Muhumuza and Gerald Grant

S pina bifida or myelomeningocele describes a class of neural tube defects (NTDs) that are caused by incomplete neural tube closure during the 4th week of pregnancy and result in various levels of neurological deficit. Adequate folic acid consumption during early pregnancy prevents the occurrence of NTDs. However, because the neural tube closes by week 4 of fetal development, early access to antenatal care (ANC) is crucial in the prevention or early detection of NTDs. 8 In Uganda, 4 routine ANC visits are recommended as part of the national clinical guidelines

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Jennifer A. Moliterno, Michael L. DiLuna, Shreya Sood, Kurt E. Roberts and Charles C. Duncan

heart murmur. Her family history was significant for a cousin with spina bifida occulta. She unexpectedly conceived ~ 1 year after her gastric bypass. Prenatal ultrasonography was initially performed for cardiac evaluation, given her other child's heart murmur and revealed an open neural tube defect and myelomeningocele at approximately L-5 to S-1. The banana sign and lemon sign were also noted along with clubbing of the right foot. Alpha-fetal protein measurement was not performed because the patient declined amniocentesis. Baseline folate and B 12 levels were

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Shenandoah Robinson, Bruce A. Kaufman, John A. Jane Jr. and Tae Sung Park

This volume of Neurosurgical Focus covers an array of topics useful to many specialists who care for patients with neural tube defects and related abnormalities. These patients have specific needs at each stage of the lifespan. Today, many individuals with significant developmental abnormalities of the spinal cord are leading full lives. For example, it is important for obstetricians and anesthesiologists to be cognizant of the underlying abnormalities associated with a lipomyelomeningocele or myelomeningocele to optimally care for a woman with one of

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Dwarakanath Srinivas, Bhavani Shankar Sharma and Ashok Kumar Mahapatra

A nencephaly , encephalocele, and spina bifida constitute the spectrum of NTDs. Triple neural tube defects are rare entities with only 1 case existing in the available literature. 7 There has been a divergent view on the etiogenesis of NTDs. 1 Two main theories have been postulated: the more widely held orderly closure theory and the less accepted multisite closure theory. In this article we present the second and third cases of a triple NTD, including the first reported triple MMC. We also explain and examine the embryological basis of NTDs with special

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Simona Mihaela Florea, Alice Faure, Hervé Brunel, Nadine Girard and Didier Scavarda

neural tube defects (ONTDs) in their most severe form or limited dorsal myeloschisis (LDM) in the lesser. 6 , 18 , 19 When the secondary neurulation process is impaired, the result is either a defect of formation that leads to an absent conus and a short spinal cord, a condition known as caudal agenesis; 15 or a defect during its regression as we can see in terminal myelocystocele, ventriculus terminalis, or lipomas of the filum terminale. 16 When there are alterations present in both the primary and secondary neurulation we can find mixed dysraphisms that present

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Harsh Deora, Dwarakanath Srinivas, Dhaval Shukla, B. Indira Devi, Ajit Mishra, Manish Beniwal, Narasinga Rao Kannepalli and Sampath Somanna

M ultiple-site neural tube defects (MNTDs) are extremely rare. Most of the cases are reported from developing nations, predominantly India or African countries. The term “multiple-site neural tube defects” refers to defects reported in a single case with “normal” neural tissue in between. It needs to be differentiated from “complex” spinal defects, as these may be many different defects (up to 5 reported) but in a contiguous manner. Thus, there may be multiple lesions, but there are not multiple sites, as these have a different embryological basis. The classic

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Craig H. Rabb, J. Gordon McComb, Corey Raffel and J. Gerald Kennedy

S pinal arachnoid cysts are an infrequent cause of myelopathy and radiculopathy and often yield gratifying results when diagnosed and treated. These cysts have been classified as either extradural or intradural. The etiology of these lesions is unclear. Familial occurrence has been noted. 1, 4, 5 Published associations include hereditary distichiasis 28, 31 and neurocutaneous melanosis, 17 but not neural tube defects. In reviewing our experience in a pediatric population, we found that the majority of spinal arachnoid cysts in our series were in patients

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Timothy W. Vogel, Calvin S. Carter, Kingsley Abode-Iyamah, Qihong Zhang and Shenandoah Robinson

N eural tube disorders are a set of complex neural developmental abnormalities that occur in approximately 1 of 1500 births 4 and are the second most common birth defect. 32 Neural tube defects include both open and closed forms—with open forms resulting in failure of neural tube closure, seen in the cranial region with anencephaly and in the spinal region with spina bifida. Occasionally the entire craniospinal axis can be involved, such as in cases of craniorachischisis. Closed forms can present with more subtle phenotypic changes that are skin covered

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Poomagame Narasimhamurthy Harisha, B. Indira Devi, Rita Christopher and Tumkur Puttasiddhappa Kruthika-Vinod

supervision: Devi, Christopher. Portions of this work were presented as a Top Ten Abstract in pediatric neurological surgery at the Congress of Neurological Surgeons Annual Meeting, New Orleans, Louisiana, October 24–29, 2009. References 1 Angeline T , Jeyaraj N , Granito S , Tsongalis GJ : Prevalence of MTHFR gene polymorphisms (C677T and A1298C) among Tamilians . Exp Mol Pathol 77 : 85 – 88 , 2004 2 Boyles AL , Hammock P , Speer MC : Candidate gene analysis in human neural tube defects . Am J Med Genet C Semin Med Genet 135C : 9

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Esther B. Dupépé, Daxa M. Patel, Brandon G. Rocque, Betsy Hopson, Anastasia A. Arynchyna, E. Ralee' Bishop and Jeffrey P. Blount

E ach year approximately 1500 babies are born in the United States with myelomeningocele, the most common form of neural tube defect (NTD). 7 According to the Centers for Disease Control and Prevention NTD data and statistics, the prevalence of NTD by ethnicity is as follows: 4.17 per 10,000 in Hispanics, 2.64 per 10,000 in non-Hispanic African Americans, and 3.22 per 10,000 in non-Hispanic whites. 27 Although the incidence of NTD has decreased during the last 20 years, possibly due to folic acid supplementation, it remains the most common