Glossary in Congenital Malformations

NeuroLearn NeuroHelp Malformations @ General Syndromes

General:

#Neuroenteric canal: During embryogenesis, the neuroenteric canal connect the neurotube with the york sac, the structure that later develop into the midgut. The neuroenteric canal should close during development. A rare malformation, resulted from persistence of neuroenteric canal will allow the content of the gut to flow into the spinal central canal and causes infection.

#Neuroenteric cyst: they are rare intraspinal and intracranial extramedullary cysts lined by epithelium resembling that of the gastrointestinal tract and are usually located at caudal cervical, and rostral thoracic levels.

#Neuropore closure: Rostral neuropore closes around 22 days of gestation. Caudal neuropore closes at around 26 days of gestation; the closure occurs at the level of future somite 31, sacral vertebra 2.

#Neurotube defect: A group of defects, heterogeneous in morphology and causes, in which the neural tube has usually failed to complete neurulation and one or more of the neural tube coverings are incomplete.

#Neurulation: The process of formation of the neural plate, the neural folds, and their closure to form the neural tube is called neuralation.                          

·         Primary neurulation: It can be divided into 4 spatially and temporarily overlapping stages: neural plate formation, neural plate shaping, neural plate bending, and closure of the neural groove.

·         Secondary neurulation: It refers to the formation of the caudal portion of the spinal cord in avian and mammalian embryos. It occurs at about the time the posterior neuropore closure and involves canalization of a mass of cells derived from the caudal cell mass. Two processes occur: formation of the medullary plate and cavitation of the plate. The lumen formed in the medullary plate by cavitation is always continuous with the lumen of the neural tube formed by primary neurulation. This process is variable among individuals. Once the spinal cord is formed the caudal portion atrophies and form the filum terminale.

#Nodular cortical dysplasia: also known as brain warts. They are nodules (1-2 mm in diameter) of cortical layers II and III protrude through a thin or absent molecular layer. Neurons of various size are grouped around a radial bundle of myelinated fibers and a central blood vessel. They are most commonly found in the frontal lobe near the operculum or at the orbital surface.

#Notochord: an axial stiffening rod extending along the length of the animal and lying immediately above the alimentary canal and below the central nervous system. It may persist throughout life, as in lower chordate animal; or be replaced, either wholly or partially, by a backbone or vertebral column, as in the higher chordate animal.

 

Syndromes and SequencesHead

NeuroLearn NeuroHelp Malformations General Syndromes For Comment: KarMing-Fung@ouhsc.edu