Neural Tube Defects (Exencephaly)

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
Networked: 3208 relevant articles (103 outcomes, 316 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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


1. Finnell, Richard H: 46 articles (09/2015 - 01/2002)
2. Shaw, Gary M: 30 articles (11/2015 - 01/2002)
3. Scott, John M: 30 articles (06/2015 - 11/2002)
4. Ren, Aiguo: 29 articles (09/2015 - 11/2003)
5. Koren, Gideon: 24 articles (06/2015 - 01/2002)
6. Molloy, Anne M: 24 articles (06/2015 - 01/2002)
7. Mills, James L: 23 articles (06/2015 - 11/2002)
8. Zhang, Le: 22 articles (09/2015 - 06/2006)
9. Copp, Andrew J: 22 articles (01/2015 - 02/2002)
10. Zhang, Ting: 21 articles (12/2015 - 01/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neural Tube Defects:
1. Folic Acid (Vitamin M)FDA LinkGeneric
2. alpha-Fetoproteins (AFP)IBA
3. VitaminsIBA
4. Glucose (Dextrose)FDA LinkGeneric
5. Valproic Acid (Valproate, Semisodium)FDA LinkGeneric
6. AcetylcholinesteraseIBA
7. Methionine (L-Methionine)FDA Link
8. Inositol (Myoinositol)IBA
9. Aspirin (Acetylsalicylic Acid)FDA LinkGeneric
10. Pantothenic Acid (Calcium Pantothenate)FDA Link

Therapies and Procedures

1. Induced Hyperthermia (Thermotherapy)
2. Catheters
3. Neonatal Intensive Care
4. 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. "
5. 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 - "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 - "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. "