Lecture 25 Embryology and Congenital Malformation Flashcards
Where does the nervous system develop from
The embryonic ectoderm
What week does the CNS appear
3rd week
Define the neural plate
thickening of ectoderm anterior to the primitive node
Define the Neural folds
Edges of the neural plate thicken and move upwards to form the neural folds
Neural fold migrate towards each other to form what?
Neural tube
Remains open at anterior and posterior ends
When does the anterior (cranial/rostral) neuropore close
Dat 25
18-20 somite stage
When does the posterior (caudal) neuropore close
Day 27
Where is neural tube closure initiated
Several points along A-P axis
When does neural tube closure begin
Day 18
When is neural tube closure completed
Day 27 (end of 4th week)
How many closure sites are there in humans
Up to 5
Failure of neural tube to close properly leads to what neural tube defects
Anencephaly
Encephalocoele
Spina bifida
How common is Anencephaly
1:1500
4 x more common in women
What causes Anencephaly
Failure of anterior neuropore to close
Skull fails to form
Brain tissue degenerates
incompatible with life
Define Craniorachischisis
Failure of neural tube closure along entire neuraxis
How common is Encephalocoele
1:4000 births
What is Encephalocoele
Herniation of cerebral tissue though a defect in the skull
Failure in closure of rostral neural tube
Where is Encephalocoele most common and where else can it be found
Occipital region
Fronto-nasal region
What is the cause of Spina Bifida
Defective closure of the caudal neural tube. Affects tissues overlying the spinal cord. Non fusion of vertebral arches
What are the types of Spina bifida
Spina bifida with meningomyelocele Spina bifida with myeloschisis Spina Bifida Occulta Spina Bifida Cystica Spina bifida with meningocele
Define Spina bifida with meningomyelocele
This is when the neural tube has failed to close and the neural tissue is exposed on the baby’s back
Define Spina bifida with myeloschisis
The nerve tissue is here fully bare and a dermal or meningeal covering is absent
Define Spina Bifida Occulta
Most minor form
Failure of embryonic halves of vertebral arch to grow normal and fuse
What are the features of Spina Bifida Occulta
No clinical symptoms
Dimple with small tuft of hair
What vertebral level does spina bifida occult occur
L5 and L6
What is Spina Bifida Cystica
Protrusion of spinal cord and/or meninges through the defect in the vertebral arches
How common is Spina Bifida Cystica
1:1000
What is Spina bifida with meningocele
Rarest form
Protrusion of meninges and CSF
What is Spina bifida with meningomyelocle associated with
Hydrocephalus
Neurological deficits- loss of sensation and muscle paralysis(loss of sensation and muscle paralysis)
What is the most severe form of spina bifida
Myeloschisis- • Spinal cord in affected area open due to failure of neural folds to fuse
When does the brain vesicles develop
Begins with closure of anterior neuropore (~day 25; 18 – 20 somite stage)
How is Spina bifida prevented
Folic acid supplements
How is Spina bifida diagnoses pre-natal
Maternal blood screening- AFP from foetal liver (16-20 weeks)
Aminocentesis (AFP in amniotic fluid)
US- anencephaly (12 weeks ) and spina bifida (16-20 weeks )
The forebrain develops from what vesicle
Prosencephalon
The Midbrain develops from what vesicle
Mesencephalon
The hindbrain develops from what vesicle
Rhombencephalon