Neural Tube Defects (Exencephaly)

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2492 relevant articles (81 outcomes, 250 trials/studies) found for this Disease

Description: Congenital malformations of the central nervous system and adjacent structures related to defective neural tube closure during the first trimester of pregnancy generally occurring between days 18-29 of gestation. Ectodermal and mesodermal malformations (mainly involving the skull and vertebrae) may occur as a result of defects of neural tube closure. (From Joynt, Clinical Neurology, 1992, Ch55, pp31-41)

Also Known As:

Exencephaly; Diastematomyelia; Iniencephaly; Neural Tube Defect; Craniorachischisis; Acrania; Developmental Neural Tube Defects; Neural Tube Developmental Defects; Neuroenteric Cyst; Acranias; Craniorachischises; Cyst, Neurenteric; Cyst, Neuroenteric; Cysts, Neurenteric; Cysts, Neuroenteric; Defect, Neural Tube; Defects, Neural Tube; Diastematomyelias; Exencephalies; Iniencephalies; Myelodysplasia, Spinal Cord; Myelodysplasias, Spinal Cord; Neurenteric Cysts; Neuroenteric Cysts; Spinal Cord Myelodysplasias; Tethered Cord Syndromes; Developmental Defects, Neural Tube; Neurenteric Cyst; Spinal Cord Myelodysplasia; Tethered Cord Syndrome

Relationship Network

Disease Context: Research Results

Nervous System Diseases: 3643

Nervous System Malformations: 21

Neural Tube Defects: 2492

Spinal Dysraphism: 868

Meningomyelocele: 491

Anencephaly: 342

Encephalocele: 168

Meningocele: 89

Arnold-Chiari Malformation: 67

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 449

Congenital Abnormalities: 10008

Nervous System Malformations: 21

Neural Tube Defects: 2492

Spinal Dysraphism: 868

Meningomyelocele: 491

Anencephaly: 342

Encephalocele: 168

Meningocele: 89

Arnold-Chiari Malformation: 67

Related Diseases

1.	Congenital Abnormalities (Deformity)
2.	Spinal Dysraphism (Spina Bifida)
3.	Neural Tube Defects (Exencephaly)
4.	Heart Diseases (Heart Disease)
5.	Neoplasms (Cancer)

Experts

1.	Suarez, Lucina: 10 articles (05/2008 - 03/2003)
2.	Finnell, Richard H: 9 articles (01/2007 - 01/2002)
3.	Li, Zhu: 8 articles (10/2006 - 01/2002)
4.	Mills, James L: 8 articles (04/2006 - 11/2002)
5.	Felkner, Marilyn: 7 articles (05/2008 - 05/2004)
6.	Vellenga, Edo: 6 articles (01/2008 - 02/2003)
7.	Drayer, A Lyndsay: 6 articles (01/2008 - 02/2003)
8.	Fuhler, Gwenny M: 6 articles (01/2008 - 02/2003)
9.	Brody, Lawrence C: 6 articles (04/2006 - 11/2002)
10.	Scott, John M: 6 articles (04/2006 - 11/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neural Tube Defects:

1.	Folic Acid (Vitamin M)FDA LinkGeneric 02/01/2009 - "Folates are important for normal neural tube closure in early gestation, and the efficacy of diet fortification with folic acid in reducing the incidence of neural tube defects is a major success story for public health nutrition. " 06/01/2008 - "A folic acid supplementation program targeting couples prior to marriage in China has led to optimal consumption of supplements containing folic acid and a significant reduction of neural tube defects (NTD). " 01/01/2009 - "When dietary modification and supplementation strategies proved inadequate, folic acid food fortification was legally mandated in the US in the late 1990s, which significantly improved population folate status and produced an abrupt decline (20%-27%) in the prevalence of neural tube defects at birth. " 12/01/2008 - "These data suggest that women of childbearing age are achieving positive folate status in the postfortification era, but it may not be sufficient to achieve red blood cell folate concentrations associated with a significant reduction in neural tube defect risk. " 08/01/2008 - "BACKGROUND: Folic acid fortification of flour has significantly decreased the incidence of neural tube defects (NTDs). " Order ALL the reference details at left...
2.	alpha-Fetoproteins (AFP)IBA 08/15/1981 - "The gel-AChE test was thus found to be very effective when applied to amniotic fluid samples with positive AFP results, substantially reducing the number of false positives with only a very small loss in the detection of open neural-tube defects. " 04/01/2002 - "Fetal sonography is effective in identifying neural tube defects, although alpha-fetoprotein screening seems to give a greater sensitivity. " 04/01/2000 - "Fetal ultrasound is effective in identifying neural tube defects, although alpha-fetoprotein screening seems to yield a greater sensitivity. " 09/01/1989 - "To assess the relative efficacy of amniocentesis versus targeted (detailed) ultrasonography, 225 patients referred because of an elevated maternal serum alpha-fetoprotein level (79.6%) or a family history of neural tube defect (20.4%) were evaluated. " 10/01/1987 - "Because the incidence of open neural tube defects is lower for blacks than for others, excessive false positive results for blacks (estimated to be 8817 to 28,215 cases annually) would be a pernicious misapplication of maternal serum alpha-fetoprotein screening. " Order ALL the reference details at left...
3.	VitaminsIBA 03/01/2008 - "CONCLUSION: Considering the high frequency of central nervous system anomalies recorded in our study, it seems to be reasonable to pay more attention to the role of periconception vitamin supplementation for the primary prevention of congenital anomalies, particularly neural tube defects." 04/01/1996 - "DESIGN: Stored blood samples collected as part of a randomised trial of vitamin supplementation in the prevention of neural tube defects were retrieved from affected pregnancies (cases) and unaffected pregnancies (controls). " 01/01/1994 - "Due to the non acceptance by ethics committees of its double blind controlled study for prevention of neural-tube defects recurrence by vitamin supplementation versus mineral components, a British group underwent methodologically debatable epidemiological study which has become quite controversial. " 01/01/1994 - "[Risk related to methodologically disputable studies: the example of vitamin therapy for the prevention of neural tube defects]" 08/01/1993 - "Recent studies have resolved the debate over the role of vitamins in preventing neural tube defects. " Order ALL the reference details at left...
4.	Methionine (L-Methionine)FDA Link 02/01/2002 - "Pre-treatment with methionine only produced a numerical but not statistically significant reduction in alcohol-induced exencephaly. " 02/01/2002 - "The low dose of methionine only numerically reduced the spontaneous exencephaly. " 02/01/2002 - "Effect of maternal methionine pre-treatment on alcohol-induced exencephaly and axial skeletal dysmorphogenesis in mouse fetuses." 10/01/2001 - "Dietary methionine is involved in the etiology of neural tube defect-affected pregnancies in humans." 11/01/1997 - "We hypothesized that women who had higher dietary intakes of methionine would be at lower risk for neural tube defect (NTD)-affected pregnancies. " Order ALL the reference details at left...
5.	Valproic Acid (Valproate, Semisodium)FDA LinkGeneric 12/01/2003 - "METHODS: In the current study, we wanted to extend our findings and test the hypothesis that Folbp2(-/-) mice are more susceptible to the teratogenic effects of valproic acid (VPA), a commonly used antiepileptic drug that is known to induce neural tube defects (NTDs) in both humans and laboratory animals. " 05/01/1997 - "The present study shows that the multifactorial genetic liability to spontaneous exencephaly in the SELH/Bc mouse strain (10-20% of embryos) also confers an elevated risk of exencephaly induced by valproic acid. " 09/01/1996 - "The objectives of this study were to determine if sodium valproate (VPA) would augment this background frequency of exencephaly and to characterize its gross and histologic bases. " 11/01/2009 - "Sodium valproate is a teratogen responsible for a wide range of abnormalities, including neural tube defects. " 05/01/2009 - "Valproate is teratogenic and specifically associated with neural tube defect. " Order ALL the reference details at left...
6.	AcetylcholinesteraseIBA 03/01/1985 - "Measurement of amniotic fluid alpha-foetoprotein and acetylcholinesterase for the detection of neural tube defects has been used in 285 normal or neural tube defects affected pregnancies: 18 false positive results (with non neural tube defects fetal malformations) have been reported. " 11/01/2001 - "Levels of amniotic fluid alpha-foetoprotein and acetylcholinesterase activity are elevated in neural tube defects. " 04/01/2000 - "Electrophoresis of acetylcholinesterase in the amniotic fluid can provide the diagnosis of open neural tube defect between 13 and 24 weeks gestation. " 01/01/1998 - "CONCLUSIONS: Raised levels of maternal serum secretory acetylcholinesterase may provide a useful indicator of neural tube defects in early pregnancy." 01/01/1998 - "BACKGROUND: To investigate the levels of maternal serum secretory acetylcholinesterase from a sample of pregnancies involving fetal neural tube defects and compare those results with alphafetoprotein levels. " Order ALL the reference details at left...
7.	Glucose (Dextrose)FDA LinkGeneric 12/01/1985 - "Although the teratogenic effects of excess glucose have been investigated in in vivo and in vivo studies, a cellular basis for neural tube defects has not been elucidated. " 01/01/2008 - "In the search for genes responsible for neural tube defects, glucose metabolism matters." 03/01/2007 - "To test whether the GLUT2 transporter is required for neural tube defects resulting from maternal hyperglycaemia, Glut2+/- mice were crossed and transient hyperglycaemia was induced by glucose injection on day 7.5 of pregnancy. " 06/01/2003 - "Neural tube defects (NTD) were found predominantly in the CDs embryos starting from 14% in the high glucose medium and increasing to 29% in the sub-teratogenic medium. " 12/01/1999 - "Evidence that elevated glucose causes altered gene expression, apoptosis, and neural tube defects in a mouse model of diabetic pregnancy." Order ALL the reference details at left...
8.	Inositol (Myoinositol)IBA 03/01/2005 - "Periconceptional dietary intake of myo-inositol and neural tube defects in offspring." 01/01/2003 - "These data can be used in further research into the association between myo-inositol and neural tube defects." 01/01/2003 - "A deranged myo-inositol metabolism has been associated with neural tube defects. " 02/01/1998 - "Dietary myo-inositol supplementation of diabetic mothers resulted in a significant decrease in the incidence of neural tube defects when compared with diabetics not receiving supplements (9.5 vs. 20.4%; P < 0.05). " 05/01/1997 - "Inositol prevents expression of a genetic model of neural tube defects in mice." Order ALL the reference details at left...
9.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 12/01/1994 - "Aspirin also reduced the frequency of alcohol-induced arched palate and the baseline exencephaly. " 12/01/1994 - "The lack of a clear dose-dependent antagonistic effect of aspirin on alcohol-induced exencephaly suggests that the production of this malformation is probably not mediated by prostaglandin as it was shown for limb and renal abnormalities (Randall, C.L., Anton, R.F. " 12/01/1994 - "Pre-treatment with aspirin (both 150 and 200 mg/kg) on day 8 of gestation resulted in a numerical, though not statistically significant increase in alcohol-induced exencephaly. " 12/01/1994 - "Effect of pre-treatment with aspirin on alcohol-induced neural tube defects in the TO mouse fetuses." 06/01/1994 - "Mechanism of aspirin induced neural tube defect in chick embryo." Order ALL the reference details at left...
10.	Pantothenic Acid (Calcium Pantothenate)FDA Link 09/01/1995 - "Pantothenic acid decreases valproic acid-induced neural tube defects in mice (I)." 03/01/2006 - "Folic acid and pantothenic acid protection against valproic acid-induced neural tube defects in CD-1 mice." 02/01/1984 - "Evidence is conflicting or inconclusive as to the use of vitamin C in the common cold, asthma and enhancement of athletic capacity, of pantothenic acid in osteoarthritis, and folic acid (folacin) in neural tube defects. " 11/01/1979 - "Optimum growth and differentiation, virtually indentical with that obtained in while serum and to that seen during the corresponding period in vivo, were obtained with the addition of glucose and certain vitamins: pantothenic acid and riboflavin had a general beneficial effect on development, i-inositol suppressed neural tube defects, and folic acid significantly improved growth of the embryos." Order ALL the reference details at left...

Therapies and Procedures

1.	Induced Hyperthermia (Thermotherapy) 06/01/2002 - "Immunohistochemical study on the fetal rat pituitary in hyperthermia-induced exencephaly." 03/01/2002 - "The risk of neural tube defects due to exercise-induced hyperthermia that is suggested by animal studies is less likely in women, because of more effective mechanisms of heat dissipation in humans. " 04/01/1986 - "In a study designed to elucidate the genetic factors involved in a mouse model of hyperthermia-induced neural tube defects, it is apparent that a hierarchy of susceptibility exists among various inbred mouse strains. " 06/01/2003 - "CONCLUSION: The data demonstrate that a single fetal genetic locus, plus a maternal effect, have likely caused the strain differences in the susceptibility to hyperthermia-induced exencephaly. " 06/01/2003 - "Genetic dissection of hyperthermia-induced neural tube defects in mice." Order ALL the reference details at left...
2.	Aftercare (After-Treatment) 10/01/1977 - "Maximum resorption (52.6%) was seen with treatment on day 7 whereas the highest rate of malformation (51.9%) was observed with treatment on day 9. Cranial defects predominated with treatment early in pregnancy (highest incidence of day 9) while limb defects were not observed with any treatment administered before day 9. Exencephaly occurred with treatments on day 6 and 8, with a single case after treatment on day 10. " 08/01/1976 - "The types of abnormality generally resembled those produced by lathyrogens in other species except for exencephaly and encephalocele, which occurred after treatment on day 7. These results indicate that betaAPN is a typical teratogen, capable of producing abnormalities of the central nervous system early in gestation, and may reflect a broader mechanism for its activity than previously suggested." 04/01/1996 - "After treatment in 6 patients bladder dysfunction improved (18%), hyperreflexia disappeared in 4 and sphinteric resistance increased in 2. In patients with tethered cord syndrome, untethering is recommended treatment in preventing further, sometimes rapid neurologic dysfunction and better results are got in childs whose condition is repaired at an early ages." 08/01/1988 - "Depending on the exact time of gestation when lithium carbonate is administered, the frequencies of exencephaly and spinal kinking after treatment 1 hour into the ninth day are compared with those after treatment 2 or 3 hours into the ninth day, incidence of exencephaly is increased at the later time and the incidence of spinal kinking is decreased." 10/01/1985 - "When treatments were given on day 9 of gestation, 500 mg/kg BUdR caused cell necrosis in the neuroepithelium about 15 hours after treatment but no neural tube defects were produced by day 9 after treatment. " Order ALL the reference details at left...
3.	Prenatal Care 03/01/2002 - "According to the American College of Obstetricians and Gynecologists, it has become standard in prenatal care to offer screening tests for neural tube defects and genetic abnormalities. " 09/01/2002 - "ALTHOUGH ADVANCES IN prenatal care and diagnosis have reduced the incidence of spina bifida, repair of neural tube defects remains one of the standard cases encountered by pediatric neurosurgeons. " 01/01/1999 - "DESIGN: Interviews (based on the Health Belief Model) concerning folate and neural tube defects were conducted in a prenatal care clinic. " 01/01/1989 - "Reliability of detecting open neural tube defects in prenatal care had been proved by quantitative analysis of acetylcholinesterase activity in amniotic fluid. " 01/16/2003 - "DEVELOPMENT: In spite of the fact that in the majority of cases of mental retardation the etiology is unknown, and for that reason, in them it is not possible to establish preventive strategies, within the last three decades, important research advances have helped to prevent thousands of cases of mental retardation of illnesses caused by Haemophilus influenzae B, measles encephalitis, Rh disease and severe jaundice in newborn infants, congenital hypothyroidism, phenylketonuria and congenital rubella; as well as removing lead from the environment, intervention programs for the proper use of seat belts, child safety seats, and motorcycle and bicycle helmets; early and adequate prenatal care, dietary supplementation with folic acid beginning before conception to reduce the risk of neural tube defects, avoidance of toxic substances during pregnancy like alcohol, and the use of newborn screening tests. " Order ALL the reference details at left...
4.	Neonatal Intensive Care 07/01/2006 - "There was a significant reduction in the proportion of deaths from chromosomal abnormalities, and from neural tube defects in epoch 2. CONCLUSIONS: There have been substantial changes in the illnesses leading to death in the neonatal intensive care unit. " Order ALL the reference details at left...
5.	Seat Belts (Seat Belt) 01/16/2003 - "DEVELOPMENT: In spite of the fact that in the majority of cases of mental retardation the etiology is unknown, and for that reason, in them it is not possible to establish preventive strategies, within the last three decades, important research advances have helped to prevent thousands of cases of mental retardation of illnesses caused by Haemophilus influenzae B, measles encephalitis, Rh disease and severe jaundice in newborn infants, congenital hypothyroidism, phenylketonuria and congenital rubella; as well as removing lead from the environment, intervention programs for the proper use of seat belts, child safety seats, and motorcycle and bicycle helmets; early and adequate prenatal care, dietary supplementation with folic acid beginning before conception to reduce the risk of neural tube defects, avoidance of toxic substances during pregnancy like alcohol, and the use of newborn screening tests. " Order ALL the reference details at left...

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