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RADIOLOGY Subject

PEDIATRIC NEURO RADIOLOGY Topic


DR. BANDONG Lecturer
2ND SHIFTING/ SEPT 25,2008 Shifting /Date
COFFEE LOVERS Trans group

PEDIATRIC NEURORADIOLOGY

STAGE 1: DORSAL INDUCTION


– Formation and closure of the Neural
Tube

• 3-4 weeks
CHIARI II
• THREE PHASES:
- Myelomeningocele
a) Neurulation
- Low tentorium
b) Canalization
- Medully kink
c) Retrogressive differentiation
- Hydrocepahalus
- Beaked tectum
ANENCEPHALY - Corpus callosum
- Failure of brain and skull development agenesis
- Most severe anomaly - Polymicrogyria
- UTZ diagnosis as early as 20 weeks - Syringomyelia
- Polyhydramnios, high alpha-fetoprotein - Lacunar skull
- Death

CEPHALOCELE
- Defect in the dura and cranium with associated
extracranial herniation felt to be related to
abnormal closure of the neural tube
- TWO TYPES:
a) Meningocele – herniation of meninges
with CSF
b) Encephalocele – herniation of brain and
meninges
- Usually midline
- Occipital location = US
- Frontal ethmoidal = Asia
- Often the herniated brain is dysgenic and
nonfunctional

CHIARI III
- Cervical occipital
CHIARI I encephalocele that
- Low tonsils contains cerebellum
- Small fourth ventricle
- Hydromyelia
- Klippel-Feil
- Occipitilization of the
atlas
- syrinx

MARY YVETTE ALLAIN TINA RALPH SHERYL BART HEINRICH PIPOY KC JAM CECILLE DENESSE VINCE HOOPS CES XTIAN LAINEY RIZ KIX EZRA GOLDIE BUFF MONA AM MAAN ADI KC
PENG KARLA ALPHE AARON KYTH ANNE EISA KRING CANDY ISAY MARCO JOSHUA FARS RAIN JASSIE MIKA SHAR ERIKA MACKY VIKI JOAN PREI KATE BAM AMS HANNAH MEMAY PAU
RACHE ESTHER JOEL GLENN TONI
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c) LOBAR
 Fusion of only
anterior
inferior frontal
lobes so no
faux in that
location
 Otherwise the
brain appears
to be quite
normal except
for lack of
septum
pellucidum

d) SEPTAL OPTIC DYSPLASIA


 Most mild form in w/c ther is no septum
pellucidum and the optic nerves are
very atrophic.
 Schizencephaly may be present in 50%
SPINAL DYSRAPHISM of these cases
 Corpus Callosum agenesis may also be
STAGE 2: VENTRAL INDUCTION seen in this entity
- Formation of the Brain segments and
Face
• 5-10 weeks
• Three vesicles (prsencephalon, mesencephalon, &
rhombencephalon) form the cerebrum, midbrain,
cerebellum, and lower brainstem
• Division into two hemispheres

HOLOPROSENCEPHALIES
- Failure to separate into hemispheres
- TYPES:
a) ALOBAR • The optic tracts are thinned bilaterally
 Complete
failure
 No falx The absent septum pellucidum
on this coronal T2 image results
 Single mono-
in a boxlike configuration of the
ventricle
anterior horn of the lateral
 Fused
ventricles. Also note the thin
thalami
optic tracts.

b) SEMI LOBAR
 Partial separation
of the posterior
occipital and
temporal lobes Coronal T1 image
 Frontal brain is demonstrates corpus
fused callosum
 Thalami partially
fused
 Ace of spades
configuration of
the ventricles
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STAGE 3: MIGRATION AND


HISTOGENESIS
CORPUS CALLOSUM AGENESIS
• Neuronal migration from germinal matrix to the
• Growth anterior to posterior starting at the genu.
cortex
Myelination from posterior to anterior
• Cortical organization
• Association with Chiari II, Dandy Walker,
• Months: 2-5
Holoprosencephaly and lipomas.
• Disorders:
• Splaying of the anterior horns (Bulls horn
o Heterotopias, agyria-pachygyria,
appearance) due to realignment of the Probst
bundles. polymicrogyria, vascular malformations,
teratomas, phakomatosis
• High third ventricle
GRAY MATTER HETEROTOPIAS
• Interruption of normal migration of neural blast
from the general matrix to the cortex
• Various degrees range from simple nodular gray
matter heterotopias to band heterotopias to
schizencephaly to lissencephaly and to
polymicrogyria.
• Seizures, mental retardation
• Association with corpus callosum agenesis,
Chiari malformations, Tuberous Sclerosis, septo-
DANY WALKER optic dysplasia.
• Defective development of the roof of the fourth SCHIZENCEPHALY
ventricle. • Gray matter extension from the ventricle to the
• Posterior fossa cyst; hydrocephalus often. cortex.
• Large posterior fossa; high torcula; absent falx in • Two types: closed lip (mild, no CSF within) and
the posterior fossa open lip (contains CSF, severe with cortical
• Partial Dandy Walker lacks high torcula. defects and large ventricles).
Represents a form of cerebellar hypoplasia • Association with septo-optic dysplasia and optic
• Mildest aspect of this spectrum would be mega atrophy.
cisterna magna.
• DDX: arachnoid cyst, mega cisterna magna

LISSENCEPHALY (AGYRIA-PACHYGYRIA)
Dandy Walker Variant • Most severe form of neuronal migrational
• Open communication of the posteroinferior 4th anomalies. Patients often have small brains,
ventricle and posterior CSF density cyst. mental retardation, spasticity, seizures.
• Large CSF density cyst occupies much of the • Agyria (complete lissencephaly) presents with
posterior fossa which is not enlarged smooth brain and is identified by figure eight
• Hypoplastic cerebellar hemispheres are “winged” configuration with clefts extending to the sylvian
anterolaterally by the cyst which stops at the fissure.
lateral angle of the cerebellar hemispheres and • Pachygyria (incomplete lissencephaly) has
does not extend anterior to them (demonstrate a broad, shallow gyre. More mild form the agyria.
hypoplastic vermis which is anteriorly and
superiorly located). Type I Lissencephaly
• Lack of gyri and alternating layers of grey and
FACIAL ANOMALIES white matter.
• With a thin outer cortical layer and
• A thicker deep layer of neurons
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• Sylvian fissures are vertically oriented and months
shallow Anterior portion of the posterior
2-3 months 4-7 months
limb of the internal capsule
Posterior portion of the posterior
4-6 months Birth-2 months
limb of the internal capsule
Anterior limb of internal capsule 2-3 months 7-11 months
Genu of the corpus callosum 4-6 months 5-8 months
Splenium corpus callosum 3-4 months 4-6 months
Central occipital white matter 3-5 months 9-14 months
Peripheral occipital white matter 4-7 months 11-15 months
Central frontal white matter 3-6 months 11-16 months
Peripheral frontal white matter 7-11 months 14-18 months
Centrum semiovale 2-6 months 7-11 months

NEUROFIBROMATOSIS 1 (von Recklinghausen)


- 1:4000 births
- Chromosome 17
- Optic nerve gliomas
- Hamartomas, gliosis
- Bone dysplasias
- Vascular dysplasias
- Neurofibromas
- Café-au-lait spots

Pachygyria development of abnormal broad and flat gyri


with abnormal shallow sulci

STAGE 4: MYELINATION
• Inferior to superior; posterior to anterior
• 5-15 months; matures by 3 years
• Failure: developmental delay, dysmyelinating NEUROFIBROMATOSIS 2
disease - Bilateral acoustic
• Myelination Milestones schwannomas
o Brain stem, cerebellum, posterior limb of - Meningiomas
internal capsule: term birth - Ependymomas
o Anterior limb internal capsule: two - Chromosome 22
months - 1:40,000 births
o Splenium of the corpus callosum: three - NF2 has less skin
months manifestations than NF1
o Genu corpus callosum: six months
o Occipital white matter
 Central: five months
(T1)/fourteen months (T2)
 Peripheral: seven months
(T1)/fifteen months (T2)
o Frontal white matter
 Central: six months (T1)/sixteen TUBEROUS SCLEROSIS (BOURNEVILLE’S DISEASE)
months (T2)
- Chromosome 9
 Peripheral: eleven months - Seizures, mental
(T1)/eighteen months (T2) retardation, adenoma
sebaceum
T1 Weighted T2 Weighted - Ash leaf spots
Anatomic Region
Image Image - Cortical tubers,
Birth – 2 periependymal nodules
Middle cerebellar peduncle Birth
months (hamartomas)
Cerebral white matter Birth – 4 3 - 5 months
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- Giant cell astrocytoma flammeus in the trigeminal nerve distribution
- Gray matter heterotopias (V1 most common), leptomeningeal venous
- Angiomyolipomas of the kidneys angiomatosis, and additional clinical symptoms
- Rhabdomyoma of the heart - The etiology is probably due to faulty
- Adenoma of the liver development of cortical venous drainage. There
- Skeletal cysts is resultant cortical hypoxia with progressive
- Lymphangiomyomatosis of the lungs atrophy and dystrophic calcification.
- The most common imaging findings are tubers, - Imaging Findings:
which are hamartomas along the subependymal o Intracranial “tram track” gyriform
surface and cortex. These lesions tend to calcify. calcifications in parietal/occipital lobes in the
middle layers of the cortex, ipsilateral to the
facial angioma, are common. These were
characteristic on plain films and are easily
identified on CT, but may not be obvious on
MRI.
o Cortical atrophy
o Skull changes include thickened diploic
space, and
enlargement
of the
VON HIPPEL LINDAU ipsilateral
- Hemangioblastomas of the brain and spine frontal sinus
- Retinal angiomas and hemangioblastomas (? o Enhancement
Retinal hemorrhages) of the
- Renal cell carcinoma angioma,
- Cysts in any visceral organ
- Pheochromocytoma

gyri, enlarged choroid


plexus and
subependymal and enlarged medullary veins
(the latter two are possibly related to
collateral venous drainage. It is unclear if the
cortical and surface enhancement is related
to the angioma alone or in combination with
cortical ischemia).

Hyperdensity is seen bilaterally in the parieto-


ocipital region in the cortex, suggesting
calcification, in a relatively symmetric distribution

Bone windows. This confirms that the hyperdense


material in the cortex is calcification.

STURGE-WEBER SYNDROME (ENCEPHALOTRIGEMINAL


ANGIOMATOSIS)
- A sporadically occurring phakomatosis
characterized by a “port-wine”vascular nevus
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MULTIPLE SCLEROSIS
• possible due to autoimmune-mediated
demyelination
• it is the most common demylenating disease
Serpiginous calcifications are • a female preponderance (20 and 40 years)
seen overlying the involved • typical location is the callo-septal interface
segment of the parenchyma. • the most typical course is a prolonged relapsing-
remitting disease.
DYSMYELINATING DISEASES • Later, the disease often shifts into a chronic-
- Alexander’s disease progressive phase
- Adrenoleukodystrophy • Iso- to hypointense on T1-weighted scans and
- Metachromatic leukodystrophy hyperintense compared to brain on T2-weighted
- Canavan’s disease scans
- Krabbe’s disease • Presence of 3 or more discrete lesions that are 5mm
or greater in size
ADRENOLEUKODYSTROPHY • Periventricular extension of these lesions into the
- Metabolic encephalopathy of childhood with deep white matter, the so called “Dawson’s finger”
widespread demyelination of white matter with is characteristic
inflammatory reaction and atrophy of the • Enhancement is variable and transient and is seen
adrenal cortex during the active demyelinating stage
- The classical form presents with onset between 5
and 10 years of age
- A sex-linked recessive inheritance pattern
- Clinical presentation is typically a young boy
previously in good health with behavior problems
and compromise of vision, hearing, and
intellectual function. Visual and hearing
impairment are characteristic features with
frequent involvement of white matter adjacent
to the medial and lateral geniculate bodies and
auditory and visual pathways.
- Radiologic features include:
o White matter involvement in the parieto-
occipitotemporal regions
o Peripheral rim enhancement along the
advancing anterior edge of the disease.
The enhancement correlates with the
active phase of the disease.
o Calcifications are infrequently identified
on CT scans
o Mass effect is not noted, but atrophy is a ALZHEIMER’S DISEASE
typical finding as the disease progresses • one of the most common causes of dementia in
o Atypical patterns of white matter the elderly
involvement have been described with • approximately 50% of the cases
predilection for the frontal region or • Neuropathologic findings for AD include senile
asymmetrical involvement of the plaques, neurofibrillary tangles, and
hemispheres granulovacuolar degeneration
• characterized by central white matter T2 • Generalized atrophy – most frequent description
abnormally posteriorly • Atrophic structural changes in the temporal lobe
and hippocampus – as characteristic of AD
• Increase Fe deposition in the cerebral cortex
(MR).
• Decreased flow and metabolic activity in
temporoparietal locations – functional imaging
studies (PET, SPECT)
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• binds reversibly to hemoglobin much more
avidly than oxygen (200-250 times more avidly)
leading to high levels of carboxyhemoglobin
• it also binds to myoglobin with an even greater
affinity than to hemoglobin
Pathology in the brain
• white matter demyelination
• edema and necrosis bilaterally in the globus
pallidus, caudate nucleus and cerebellum
Clinical Findings
• may be subtle and suggest merely a viral illness
• headaches, confusion, chest pain, nausea &
Generalized ventricular enlargement and prominence of vomiting, drowsiness, memory impairment,
the sulci agitation
PARKINSON’S DISEASE • cherry red skin is not often present
• chronic progressively disabling disease that falls
under the heading of akinetic-rigid syndromes
• a clinical syndrome, Parkinson’s disease is
clinically evident by its triad of bradykinesia and
hypokinesia, resting tremor and increased
tonicity of voluntary musculature and loss of
postural reflexes
• 1 in 100 individuals over the age of 60
• Only a slight male predominance
• No cure for Parkinson’s disease
• Current treatment include anticholinergic and
dopaminergic medications NECT shows bilaterally symmetrical low attenuation
• If left untreated, Parksinson’s disease progresses lesions in the cerebellum, globus pallidus and caudate
to frank deterioration of all brain functions and nuclei
total disability. Consequently, these loss of
functions may result in early death.

Imaging:
• nonspecific atrophy with enlarged lateral
ventricles and widened sulci on CT
• On MR, decreased width of the parts compacta
between the pars reticularis and the red nucleus
may be evident

SPECIAL THANKS KE VICTORIA RAMOS PATI


SHARLYN PATIGAS 

Area of decreased width of the low signal intensity in the


pars compacta within the substancia nigra

CO POISONING
• most fatalities result from fires, malfunctioning
stoves, exhaust systems, heaters, suicide
attempts
• impairs oxygen delivery and has its most lethal
effects on organs requiring high levels of oxygen,
i.e. the brain and heart
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