7. CNS Development and Atypical Development
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48 Terms
When does the embryonic period occur in humans?
From conception through the 8th week of gestation.
What is established by the end of the embryonic period?
Rudimentary structures of the brain and CNS.
What is gastrulation?
The process by which the embryo becomes a three-layered structure by the 3rd week.
Which region of the embryo becomes the head?
The rostral end.
What are neuro-ectodermal stem cells?
Progenitor cells that give rise to the brain and CNS.
What is the neural plate?
The region containing neuro-ectodermal stem cells.
When does neural tube development begin?
Around day 21 of gestation.
What is the first sign of neural tube development?
Formation of ridges along the neural plate.
How does the neural tube form?
Ridges rise, fold inward, and fuse into a hollow tube.
Where does neural tube fusion begin?
In the center, progressing rostrally and caudally.
When does the anterior neuropore close?
Day 25 of gestation.
When does the posterior neuropore close?
Day 27 of gestation.
What is the ventricular zone?
The layer of neural progenitor cells lining the neural tube's hollow center.
What do rostral neural progenitor cells form?
Cerebral hemispheres.
What do caudal neural progenitor cells form?
Hindbrain and spinal column.
What are the three primary brain vesicles?
Prosencephalon, mesencephalon, and rhombencephalon.
What does the prosencephalon become?
Cerebral hemispheres and thalamus.
What does the mesencephalon become?
Midbrain.
What does the rhombencephalon become?
Pons, cerebellum, and medulla.
What is a “lissencephalic” brain?
A smooth brain lacking gyri and sulci.
When does the longitudinal fissure begin to form?
Around 8 weeks gestation.
When do primary sulci begin to form?
Between weeks 14 and 26.
When does most neuron production and migration occur?
In utero.
How long do glial progenitor cells continue to develop?
Through childhood.
What are the major categories of CNS malformations?
Neural tube defects, cleavage defects, migrational defects, proliferation defects, cerebellar malformations.
What causes neural tube defects?
Failure of the neural tube to close properly.
What are examples of neural tube defects?
Spina bifida, meningocele, and myelomeningocele.
What is a cleavage defect?
Failure of the forebrain to properly divide into two hemispheres.
What is an example of a cleavage defect?
Holoprosencephaly.
What is a migrational defect?
Abnormal neuronal movement to the cortex during development.
What are examples of migrational defects?
Lissencephaly, double cortex, schizencephaly, porencephaly, agenesis of the corpus callosum.
What is lissencephaly?
A smooth brain surface due to failed neuronal migration.
What is schizencephaly?
A cleft or gap in the cerebral hemispheres.
What is agenesis of the corpus callosum?
Absence of the corpus callosum due to disrupted formation.
What is a neuronal proliferation defect?
Abnormal production of neurons.
What are examples of proliferation defects?
Megalencephaly and microcephaly.
What is megalencephaly?
An abnormally large brain.
What is microcephaly?
An abnormally small brain.
What are cerebellar malformations?
Structural abnormalities in the cerebellum.
What are examples of cerebellar malformations?
Chiari malformation and Dandy-Walker malformation.
What is the clinical goal when working with children with CNS malformations?
To support the child’s development and quality of life.
Why is family-centered care important in CNS malformations?
Families need emotional support, education, and guidance through complex care decisions.
What is a key part of clinical care for CNS malformations?
Helping families understand prognosis and available interventions.
Why is interdisciplinary care important in CNS malformations?
These conditions often involve motor, cognitive, and systemic complications.
What should clinicians consider beyond diagnosis in CNS malformations?
The family’s needs, values, and psychosocial context.
How should clinicians approach discussions of rare or complex CNS malformations?
With empathy, clarity, and honest communication.
What are important long-term goals in children with CNS malformations?
Promoting function, communication, and inclusion in daily life.
Why is early intervention critical in developmental brain disorders?
It maximizes neuroplasticity and improves outcomes.