CNS development

Congenital defects and the Embryonic Stages

Rostral nauuna ang closure then nahuhuli yung caudal

I. Neural tube defect

a. Aka as dysraphisms
b. Most common congenital anomalies of CNS
c. Some cases, just an abnormal maternal state
d. Exposure to radiation even before conception

II. Anencephaly
Spinal cord is very thin reflecting the absence of cerebral hemishphere
Infants are either still born or buhay for several days, usually die in a few days

III. Encephalocele
Magiging microcephalic sya kasi nag herniate na
Herniation of meninges
Among Asians, usually frontal naso ethmoidal ang madalas na nakikita. In general, majority
are occipital in location. Pwede parietal, or nasopharyngeal (nasa likod lang ang mass, wala
sa mukha but the patient will have frequent respiratory problems.
If frontal, they may go to orbit, lalabas yung mata so may proptosis
Treatment is complicated esp if mass is naso frontal or naso ethmoidal.

IV. Spinal Dysraphism

Spina Bifida Occulta
Midline vertebral body defect but no protrusion of spinal cord and meninges
Clue: ectoderm derived
Level of L5 and S1
No protrusion, no abnormality of SC and meninges, no symptoms usually
Usually, there are Other developmental anomaly such as syringomyelia or
tethered cord (if nakasabait yung cord, dun sila nagiging symptomatic)
A dermal sinus may be present (may canal, may connection. Minsan lumalabas
yung CSF jan. You have to excise or repair otherwise there will be recurrent
meningitis. So excise and isara) Take a look kasi minsan tuloy tuloy sya na butas
hanggang sa sub arachnoid space
Deviated luteal cleft.

Meningocele’
 Sac like structure with CSF. Meningeas herniate through the defect of
the post vertebral arch
If skin overlying the mass is very thin, surgery din immediately after birth.
In utero, if Makita na maydroon nito, CS na yan.

Myelomeningocele- kasali na ang sc pag herniate including the cauda equine. Most severe form
of spinal dysraphism. The px wil manifest with multi organ dysfunction. Bc lower SC supply distal tract, gi
and genital tract and lower estremities. Defect may be asct with hydrocephalus. Image the brain. Chiari
2- meningomyelocle plushydrocephalus. Treatment is multidisciplinary. Early on, UTZ the kidney, check
renal punction. Then ortho because club feet may also occur, also limb length discrepancy ( mas mahab
yung isa), rehab, ortho, nephron, uro.

Chiari 2- practically present in al meningomyelocele. Symptoms: lower brainstem symproms. CN

9, 10, 11, 12- innervates it so they’sre affected. On imaging, di makikita foramen magnum kasi itim yung
bone in MRI. Then cerebellar tonsil herniates. Lower brainstem looks like ribbon. Stripe fat kasi scalp
may loose areolar and diplo may marrow fat also so white sya.

Abnormalities of development of cerebral hemishpers”:

1. Lissencephaly (Agyria)
Absence of convolution, sylvian fissures may not be present or poor. Brain has appearance
of 4 layer instead of 6, looks like melon.…Nagkasakit ang nanay at 4 months. Has intractable
seizures, developmental delay and mental retardation

Polymicrogyria
Daming kulubot. Poorly developed gyri. Manifest with severe mental retardation.
Microcephaly.

Schizencephaly
A lot of slits. Pwedeng unilateral or bilateral ( tuloy tuloy na hanggang sa lateral ventricles).
Manifestation include seizures, mental retard and microcephaly.

Spasticity- white matter syndrome

Seizure and –mental retardation- gray matter syndrome

Porencephaly
Usually destructive. Cysts are usually lateral and usually don’t communicate with the
ventricle. Parang may communication pero there could be a very thin membrane. No b rain
tissue, may necrosis, fluid yung papalit.

Holoprosencephaly
From defective cleavage. Hindi nahati yung prosencephalon. May lobar, semilobar, may
konting thalami. No partition. Single ventricle and mortality is high. Cyclops., or magkalapit
yung eye. “the face is the window to the brain”. But eyes yung totoong window to the brain
:P

Agenesis of the corpus callosum

Could be complete or patial. CC is the major white matter ….
CC is small. The rest hindi ng develop. In effect, 3rd ventricle nag hherniate pataas. Parang
may 3rd ventricular cyst sila in effect. My low IQ, may MR. Subtle manifestation lang. May
Synckinesis. Pag nagsusulat, gumagalaw din sila.Usually soft neurologic signs

Hydranencephaly
Falx cerebri only. The rest puro nalang tubig. Left and right thalami.
May be inborn. Like congenital absence of cerebral hemisphere kasi hindi nag develop yung
internal carotid. So hindi rin mag susurive yung cerebral hemisphere so matutunaw sila,
tubig yung papalit.
Agenesis of internal carotid-may thalami, intact brainstem kasi blood supply of brain is nasa
posterior circulation (structures supplied are intact here).
In utero severe hydrocephalus- naipit na yung blood supply so here’s full blow
hydranencephaly

HEAD SIZE ABNORMALITIES

Some are congenital some acquired

Hyrdrocephalus
Inc head size plus head pressure. Mayron Malaki lang kasi ang ulo pero hindi hydrocephalic.
Enlarged ventricular cavities bc of inc pressure.
Usually due to inc CSF production or impaired absorption of CSF. But majority is impaired
absorption. Choroid plexus papilloma causes inc CSF production (which is bihira lang sa
hydrocephalus)
Can be communicating or non communicating. Obstructive is not used anymore.
Communicating- at level of arachnoid granulations or villi.
Non communicating- obstruction inside ventricular system

Yung lumalabas sa Mejendi, hindi nag tutuloy2 sa SPical canal. Kasi at birth, Spinal canal has
no CSF …. (sorry not clear)
CSF producing- roof of 3rd ventricle, floor of lateral ventricle, (mainly silang dalawa) and
konti sa 4th ventricle.
Lateral ventricle- dumadaan sa Monro. Then pupuntang 3rd ventricle. Dadaan sa cerebral
aqueduct (or Sylisan) then 4th ventricle, then labas sa Luschka and then Majendi, then sa
subarachnoid space. Pwedeng bumaba and then upwrds na sila.
REVIEW CSF CIRCULATION
Sinuses- mainly dura (double layer-endosteal andmeningeal) kung saan nag hihiwalay,
andun yung venous sinus. Dun pumapasok ang CSF sa arachnoid villi.
Kdalasan ng communicating is from meningitis, subarachnoid space, less frequent tumors.
(leptomeningeal tumor)
Non communication-any obstruction of the ventricular system

But if closed na yung sutures, hindi na rapidly inc head size.

Cranial MRI- upper motor or problem in central

EMG or CV- lower motor neuron, brainstem or CN, or SC or peripheral nerve problem
Center for voming- brainstem (saan sa brainstem)

Not all ataxia are cerebellar or post sc problem . some are frontal ataxia
Brain can accommodate with no symtpms in severe hydrocephalus esp in closed fontanels
na

Post meningitc and post hemorrhagic- most common cause of communication

hydrocephalus
Infectious aqueductal stenosis-may be caused by measles, german measle, pwedeng
mabara yung cerebral aqueduct causing non communicating hydrocephalus

If >2 y.o. less or almost no progressive head enlargement but symptoms of inc ICP na.
Ventriculoperitoneal shunt- drains in abdomen

Cocktail Party syndrome-maiingay sila, matitinis yung boses, kung ano ano sinasabi.

Surgery for rapidly enlarging head size and symptomatic patient.

Pwedeng benign subarachnid enlargement lang- give acetazolamide

Dandy –Walker Syndrome

Congenital problem, may triad: hydroceph, 4th ventricular cyst and cerebellar hypoplasia
May be asstd with other inborn anomalies

Microcephaly
Head size is <2SD below mean

Craniosynostosis
Premature closue of cranial sutures. Maliit ang ulo but configurations are irregular. Minsan
matulis or flat.