CEREBRAL

CONGENITAL
ANOMALY

Congenital
migrational disorders

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

hydranenc

lissencephaly

Polymicrogyria
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

A 10-month-old female with developmental delay, microcephaly,

and seizures presents for evaluation

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

schizeocephaly

Heterotopic gray matter

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-yearold 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.

Sagittal T1 weighted image

demonstrates large midline
arachnoid cyst and partial
agenesis of the corpus
callosum

Axial T2 weighted image

demonstrates bilateral bundles of
gray matter in the centrum
semiovale. The arachnoid cyst is
present in the midline.

Diagnosis: Band Heterotopia

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

polymicrogyria

Holoprosencephaly
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, suggesti
ve 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

Cephaloceles are congenital herniations of intracranial structures

through a skull defect