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
Sagittal T1 weighted image
demonstrates bilateral bundles of
demonstrates large midline
gray matter in the centrum
arachnoid cyst and partial
semiovale. The arachnoid cyst is
agenesis of the corpus
present in the midline.
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 ventriculo
megaly

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