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Neural Tube Defects (NTDs)
Published in Charles Theisler, Adjuvant Medical Care, 2023
During the embryo's development, a structure called the neural tube will later become the spinal cord, brain, and spinal column. Neural tube defects (NTDs) are a group of birth defects in which an opening in the spine or cranium from the first month of development remains open. This results in a hole somewhere over the spine (spina bifida) or anencephaly in a developing fetus.1 Many affected individuals do not survive. Females are more likely to be affected by NTDs than males. These defects occur in the first month of pregnancy, before a woman even suspects or knows that she is pregnant.
Developmental Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James H. Tonsgard, Nikolas Mata-Machado
Encephalocele is a herniation of intracranial contents including the brain and meninges. It is a mass protruding from the skull, most commonly in the occipital area (Figure 9.6). Frontal encephaloceles are much less common, but are more frequent in the Asian population. Basal defects involving the sphenoid or ethmoid sinuses also are seen. The amount of herniated neural tissue in the defect is variable and in part determines severity of the deficits. The cause of encephaloceles is unclear although they are clearly defects in closure of the anterior neural tube. Nasofrontal lesions are thought to be due to defective separation of the neural and surface ectoderm at the site of closure of the anterior neuropore. Occipital encephaloceles may be due to defective segmentation of the bones of the posterior cranium.
Paper 3
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Myelomeningocele is a type of spina bifida; not only are the meninges and cerebrospinal fluid present in the neural tube defect, but neural tissue too. This does not involve any splitting of the spinal cord. Syringomyelia, or syrinx, is a collection of fluid centrally in the cord and often occurs secondary to other conditions such as myelomeningocele, Chiari or Dandy-Walker malformations.
Selenium and L-carnitine protects from valproic acid-Induced oxidative stress and mitochondrial damages in rat cortical neurons
Published in Drug and Chemical Toxicology, 2022
Ahmad Salimi, Nasrin Alyan, Nasim Akbari, Zhaleh Jamali, Jalal Pourahmad
Oxidative stress has been implicated in the mechanisms of cytotoxicity and teratogenesis of VPA (Chateauvieux et al. 2010). The well-known fetal valproate syndrome, spina bifida is a neural tube defect (NTD) that results from failure of the caudal neural tube to close during organogenesis after exposure with VPA. The brain and the spinal cord develop from the neural tube through the process of neurulation during organogenesis (Tung and Winn 2011). In the current study, we chose the neonatal rats for isolation of neurons due to previous studies have been suggested that young children (less than 7.5 years) have an increased risk of toxicity of valproic acid (Star et al. 2014). Our results showed that valproic acid is toxic for isolated rat neurons up to 50 µM. Previous studies have shown that the therapeutic and toxic concentrations range for valproic acid is 40–100 and 100–200 mg/L respectively (Schulz et al. 2012). Therefore, used concentrations in this study are in therapeutic and toxic range of valproic acid (Figure 1). Interestingly, some studies have been shown neuroprotective effect of valproic acid, therefore this has a controversial effect with our results (Ximenes et al. 2015, Silva et al. 2018). However, the mentioned effect as protective have been shown in adult animals and neurodegenerative disease models, while our results obtained from in vitro models in isolated neurons.
Efficiency of topical rifampin on infection in open neural tube defects: a randomized controlled trial
Published in International Journal of Neuroscience, 2021
Ibrahim Deger, Murat Başaranoğlu, Nihat Demir, Abdurrahman Aycan, Oğuz Tuncer
Patients with neural tube defect may experience hydrocephalus, neurogenic bladder, superficial, and systemic infections, complications due to VP shunt (dysfunction and/or infection), or orthopedic problems in different periods of their life after birth (5). So, they need to be treated with a multidisciplinary approach throughout their lives (1). With a decrease in complications associated with infections, survival rates in NTD have increased (5). In this randomized controlled study, the efficiency of topical Rifampin on infection in open neural tube defects was investigated. To the best of our knowledge, this is the first randomized controlled trial on topical Rifampin use in paraplegic open neural tube defects. Also, the similar demographic characteristics of our sample and the presence of a standard protocol give greater value to our findings.
Craniorachischisis with Exencephaly
Published in Fetal and Pediatric Pathology, 2021
Jessenia Guerrero, Debra S. Heller, Ada Baisre de Leon
Fetuses with craniorachischisis are characterized by anencephaly, however, occasionally exencephaly, which is recognized by the protruding brain tissue and absence of a calvarium [4], can be seen. The majority of cases of severe open neural tube defects present as anencephaly, with only a small fraction presenting with a disorganized, malformed brain tissue, which is exposed. They are both thought to be opposite ends of the most severe open neural tube defect, with most cases starting as exencephaly and the brain tissue dissolving while exposed to the amniotic fluid, resulting in the most common presentation of anencephaly [5]. Our case is very well preserved with both craniorachischisis and exencephaly, which was diagnosed as anencephaly by sonogram, with the classic microscopic appereance of exencephalic brain, showing ependymoplastomatous rosettes and haphazardly arranged inmature neural tissue [6].