PEDIATRIC NEUROLOGICAL

DISEASES: CEREBRAL ANOMALIES

Basic diagnostic modality
MRI
US

CT has been left away because of its
radiation exposure & MRI is superior in
imaging the specific pediatric problem
Main group CNS malformations
Dysgeneses
Dysraphias
Sulcation migration disorders
Brain volume disorders
Hydrocephalus
Phakomatoses
Dysgeneses
Proencephalic vesicles defective
development
Non-disturbed neural tube closure
e.g.: Acephalia, holoprosencephaly
Holoprocencephaly
Dysraphias
Defective neural tube closure
Cranial dysraphias
Rhombocerebellar dysraphias
Dysraphias
Cranial dysraphias
Anencephaly
Rhombocerebellar dysraphias
Meningocele
Cerebellar hypoplasia
Dandy-Walker syndrome
Arnold-Chiari malformation
Aquaduct stenosis
Encephalocele
Meningocele
CSF
Encephalocystomeningocele
Cystic rudiment ventricle + brain parenchyma +
CSF
Encephalocele
Brain parenchyma
Encephalocystocele
a ventricle-connected CSF cavity + brain
parenchyma
Meningocele Encephalocystomeningocele
Encephalocele Encephalocystocele
Arnold-Chiari Malformation
[Classification Based on deformity severities]
Type I
Herniation of cerebellar tonsil & inferior
cerebellum through foramen magnum
Syringomyelia
Type II
Type III
Type II + occipital encephalocele
Arnold-Chiari Malformation
Type I
Herniation of
cerebellar tonsil &
inferior cerebellum
through foramen
magnum
Syringomyelia

Arnold-Chiari Malformation
[Based on deformity severities]
Type II
Deformation of the brain stem
Herniation of tonsils & inferior vermis through
foramen magnum
MO elongated & overlaps the upper segments of
cervical spinal
Meningomyelocele, Hydrocephalus
Expanded spinal canal, enlarged & oval-formed
foramen magnum
4
th
ventricle at the level of foramen magnum
Hypoplasia of the falx & tentorium
Cerebelum is displaced supratentorial between the
posterior horns of lateral ventricle
Arnold-Chiari Malformation type II
Dandy-Walker Syndrome
Triad:
Inferior vermis aplasia/hypoplasia
& displacement of the hypoplastic
cerebellum (laterally)
4
th
ventricle cystic transformation
Hydrocephalus
Dandy walker malformation
Frequent cause of the
congenital hydrocephalus
CT / MRI view: the first 3
ventricles are distended
while the 4
th
ventricle
remains normal
Craniosynostoses
Congenital Aqueduct Stenosis
Phakomatoses
Dysplasias with disturbed cellular migrations
1. Sturge-Weber syndrome
Encephalotrigeminal angiomatosis
CT/ MRI : Calcification on cortical regions;
leptomeningeal angiomatosis of the
temporal, parietal, occipital; atrophy
CT/ MRI + contrast medium: diffuse
enhancement at the margins of calcium
deposits
Sturge Weber syndrome
Phakomatoses
Dysplasias with disturbed cellular migrations
2. Neurofibromatosis
3. Tuberous Sclerosis
4. Hippel-Lindau syndrome

Migration
Schizencephaly
Lissencephaly / Agyria & Pachygyria
Polymicrogyria
Schizencephaly
Slit-shaped defects along the
fissures (sylvian)
Stretch from ventricular wall
up to cerebral membranes of
cortex
Limiting gray matters with
abnormal stratification
Aplasia of the pellucid
septum
Lissencephaly
(Agyria)
Telencephalon remains in a
primitive form wo/ developing
convolutions
Failure to form sulci
Abnormal development of the
cerebral fissure
Widening of the cortex
Pachygyria

Hydrocephalus
Congenital hydrocephalus, the most
common frequent pediatric neurological
condition (0.3% of all newborn)
Imbalance between the production &
absorption Increase amount of CSF (N:
150 ml) ICP & brain parenchyma
pressure gradient raised
CSF Physiology
CSF Production: 80-90% by choroid plexus.
Daily secretion rate of 500 ml.
CSF Absorption: Arachnoid granulations above
the cerebral hemispheres
CSF Flow: Lateral ventricle Foramen of
Monroe 3
rd
ventricle Sylvian aqueduct
4
th
ventricle Foramen of Magendie &
Foramina of Luschka cerebello-medullary
cistern (cisterna magna) & Spinal subarachnoid
space
Obstructive Hydrocephalus
(non-communicating)
Obstruction of the passage of the
ventricular system due to:
Congenital malformation: aqueduct stenosis,
Dandy-Walker syndrome, Arnold-Chiarri
malformations
Mass/tumor lesion
Congenital inflammation (TORCH)
Perinatal ventricular hemorrhages

Obstructive Hydrocephalus
(non-communicating)
Localized by the pattern of the
ventricular enlargement
aqueduct stenosis
Communicating
Hydrocephalus
CSF production exceeds the
absorption
30% of the hydrocephalus
cases
Less severe ventricular
enlargement, slight
involvement of 4
th
ventricle,
widening of the basal cistern
Treated Hydrocephalus
VP Shunt
Effective drainage, ventricular volume
decreased, restoring its own structure
Permanent parenchymal damage: form of
cerebral atrophy with widened cistern &
sulci
REFERENCES
1. Grumme T, Kluge W, Kretzschmar K, Roesler A. Cerebral and
Spinal Computed Tomography 3
rd
Edition. 1998. Blackwell: Berlin.
2. Francis A Burgener, Diffrential Diagnosis in Magnetic Resonance
Imaging, 2002. Georg Thieme Verlag , Stuttgard Germany.