Neuronal migration disorders

Neuronal migration disorders (eg, lissencephaly, or loss of sulci) are caused by the abnormal migration of nascent neurons within the brain. A spinal mass would not be seen.

begins with formation of the neural plate. During 3rd week of fetal development.

the lateral ends of the neural plate elevate to become neural folds that fuse in the midline to form the neural tube. Fusion starts in the cervical region and extends rostrally and caudally to the anterior and posterior neuropores, respectively.

Types of congenital malformations

-deformation -disruption -dysplasia

Mechanical forces alter shape/position of a structure that was previously developing normally Clubfoot( planter flexion and internal rotation) from uterine compression ( breech or fibroids)

Destruction of a structure that was previously developing normally Amniotic band

Abnormal organization of cells within a tissue (often genetic) Skeletal dysplasias (eg, achondroplasia)+ Osteogenesis imperfects

Osteogenesis imperfecta

a skeletal disorder characterized by brittle bones that fracture easily, occurs with dysplasia of connective tissue due to absent or abnormal type I collagen.

Defect in organogenesis (eg, genetic, teratogenic) Spina bifida Holoprosencephaly( trisomy 13, fetal alcohol syndrome and retention acid, sonic hedgehog Cleft lip and palate

multiple anomalies without a known unifying cause that occur together more frequently than chance alone would predict. Vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL)

fatal condition in which the skull and meninges fail to form. The fetal brain is then exposed to amniotic fluid, resulting in degeneration of the developing forebrain

disruption to its development may affect the CNS, gastrointestinal, or genitourinary tract. CNS structures related to notochord development include the spinal cord and vertebral column; defects would not affect the scalp and skull.

CNS abnormalities Range from asymptomatic to seizures, severe intellectual disability, death Midface abnormalities Range from mild hypotelorism and cleft lip/palate to cyclopia, primitive nose, facial cleft

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Fenestrea	Answer
Fenestrae are small pores within endothelial cells that allow free fluid exchange between the intravascular and extravascular spaces. They are found in capillaries perfusing the intestine, renal tubules, and endocrine glands.	Fenestrae
Neuronal migration disorders	Neuronal migration disorders (eg, lissencephaly, or loss of sulci) are caused by the abnormal migration of nascent neurons within the brain. A spinal mass would not be seen.
Primary neurulation	begins with formation of the neural plate. During 3rd week of fetal development.
Neural plate	the lateral ends of the neural plate elevate to become neural folds that fuse in the midline to form the neural tube. Fusion starts in the cervical region and extends rostrally and caudally to the anterior and posterior neuropores, respectively.
Types of congenital malformations	-deformation -disruption -dysplasia
1-Deformation	Mechanical forces alter shape/position of a structure that was previously developing normally Clubfoot( planter flexion and internal rotation) from uterine compression ( breech or fibroids)
2- Disrubtion	Destruction of a structure that was previously developing normally Amniotic band
3- dysplasia	Abnormal organization of cells within a tissue (often genetic) Skeletal dysplasias (eg, achondroplasia)+ Osteogenesis imperfects
Osteogenesis imperfecta	a skeletal disorder characterized by brittle bones that fracture easily, occurs with dysplasia of connective tissue due to absent or abnormal type I collagen.
4-Malformations	Defect in organogenesis (eg, genetic, teratogenic) Spina bifida Holoprosencephaly( trisomy 13, fetal alcohol syndrome and retention acid, sonic hedgehog Cleft lip and palate
What is association	multiple anomalies without a known unifying cause that occur together more frequently than chance alone would predict. Vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL)
Anencephaly	fatal condition in which the skull and meninges fail to form. The fetal brain is then exposed to amniotic fluid, resulting in degeneration of the developing forebrain
Open neural tube defects caused by	Impaired primary neurulation
Notochord	disruption to its development may affect the CNS, gastrointestinal, or genitourinary tract. CNS structures related to notochord development include the spinal cord and vertebral column; defects would not affect the scalp and skull.
Holoprosencephaly	CNS abnormalities Range from asymptomatic to seizures, severe intellectual disability, death Midface abnormalities Range from mild hypotelorism and cleft lip/palate to cyclopia, primitive nose, facial cleft

Created by: Hibanj

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