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lissencephaly

pachygyria
schizeocephaly
heterotopic gray matter
polymicrogyria
holoprosencephaly
anomalies of the corpus callosum
hydranencephaly
Dandy-Walker malformations
Chiari malformations
Cephaloceles
Neurocutaneous syndromes
Vein of Galen malformation
Aqueductal stenosis
The name Polymicrogyria broken down
describes its characteristics "many small folds
in the surface of the brain".
It is also characterized by shallow sulci, a
slightly thicker cortex, neuronal heterotopia
and enlarged ventricles. When many of these
small folds are packed tightly together, PMG
may resemble pachygyria (a few "thick folds" -
a mild form of lissencephaly) in parts of the
brain
Polymicrogyria/pachygyria
An axial T1-WI ( Fig. A ) demonstrates
marked enlargement of the lateral
ventricles, as well as prominence of the
subarachnoid spaces. The cortex is
diffusely abnormal, without normal
formation of sulci and gyri. In addition,
multiple puncate hyperintensities
(arrowheads) are noted around the
ventricular margins
the diffuse cortical abnormality to better advantage, and shows a pattern
of multiple small sulci and gyri suggesting polymicrogyria. The white
matter is diffusely abnormally hyperintense. Multiple punctate
hypointensities are noted along the ventricular surface
cerebellar hypoplasia.
Lobar Holoprosencephaly
CC: Delayed motor development.
unshunted hydrocephalus, Dandy-Walker Malformation
Aqueductal Stenosis
Heterotopias
Migration of the neuroblasts from the periventricular region to
the pia usually happens during 7th to 16th week gestation.
Damage to the radial glial fibers which direct migration is
thought to disrupt this process.
Heterotopia is disorganized brain tissue, usually gray matter, in
the wrong location.
Two types: Nodular and Band
Nodular: periventricular or subcortical, Signal same as gray
matter, no enhancement
Band: close to cortex, sx: developmental delay, seizures, double
cortex sign--thin interface of white matter between band and
cortex
Clinical Presentation: An 11-year-old patient
with attention deficit disorder with new onset
of seizures presented for imaging.
Radiographic Findings: The sagittal images
show partial agenesis of the corpus callosum
and a large arachnoid cyst. In the centrum
semiovale bilaterally there are large bands of
heterotopic gray matter. These show signal
characteristics identical to gray matter on T1,
T2 and FLAIR images.
Axial T2 weighted image demonstrates
Sagittal T1 weighted image
bilateral bundles of gray matter in the
demonstrates large midline
centrum semiovale. The arachnoid cyst is
arachnoid cyst and partial
present in the midline.
agenesis of the corpus callosum
Gray matter heterotopia are collections of
nerve cells in abnormal locations secondary to
arrest of radial migration of neurons.
Heterotopia can be isolated or, as in this case,
associated with other structural anomalies.
Patients with heterotopic gray matter almost
always present with seizure disorders [13].
Heterotopia is often divided into three
groups: subependymal heterotopia, focal
cortical heterotopia and band heterotopia
Holoprosencephaly (HPE) is a structural
anomaly of the brain in which the developing
forebrain fails to divide into two separate
hemispheres and ventricles.
The forebrain (prosencephalon) incompletely
cleaves into right and left hemispheres, and
into the olfactory and optic bulbs and tracts
Phenotypes of HPE identified in a continuum of brain
malformations include the following:
Alobar HPE, the most severe, in which there is a single ventricle
and no separation of the cerebral hemispheres
Semilobar HPE, in which the left and right frontal and parietal
lobes are fused and the interhemispheric fissure is only present
posteriorly
Lobar HPE, the mildest, in which most of the right and left cerebral
hemispheres and lateral ventricles are separated but the most
rostral aspect of the telencephalon, the frontal lobes, are fused,
especially ventrally
Middle interhemispheric fusion variant (MIHF or
syntelencephaly), in which there is failure of separation of the
posterior frontal and parietal lobes, with varying lack of cleavage of
the basal ganglia and thalami, and absence of the body of the
corpus callosum but presence of the genu and splenium of the
corpus callosum. It is debated whether MIHF is part of the HPE
spectrum or a separate entity [Barkovich & Quint 1993].
This fetus has:
A) Hydranencephaly
B) Holoprosencephaly
C) Aqueductal stenosis with
ventriculomegaly
D) Normal position of choriod plexus
This image demonstrates:
A) Colpocephaly, consistent with
agenesis of the corpus callosum
B) Ventriculomegaly, suggestive
of aqueductal stenosis
C) Ventriculomegaly and a "lemon
sign", suggesting the presence of
spina bifida
D) Prominent ventricles which are
within normal limits
This coronal image of the
fetal chest demonstrates the
following:
A) Diaphragmatic hernia
B) Hydrops
C) Cystic adenomatoid
malformation
D) Cystic hygroma

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