TRANSMANTLE

Focal cortical dysplasia, type II

(transmantle dysplasia)
Focal cortical dysplasia (FCD), fi rst described by Taylor et al. in 1971, is a
common cause of intractable childhood epilepsy.

In 2011, the International League Against Epilepsy (ILAE) reclassifi ed the

disease into three tiers:

FCD type I refers to isolated cortical dyslamination in the radial (type Ia),
tangential (type Ib), or both (type Ic) directions.

FCD type II involves dysmorphic neurons, either without (type IIa) or with
(type Iib) balloon cells.

FCD type III occurs adjacent to a principal lesion such as hippocampal

sclerosis (type IIIa), tumor (type IIIb), vascular malformation (type IIIc), or other
acquired conditions such as trauma/ischemia/infection (type IIId).
 TECTAL
BEAK/POINT/SPUR
 Chiari II malformation

The Chiari II malformation is a complex

congenital anomaly consisting of small
posterior fossa, inferior displacement of
the cerebellum and brainstem, and
lumbosacral myelomeningocele.
TEARDROP
 Callosal agenesis

Dysgenesis of the corpus callosum is caused by abnormalities in

neural development between 12 and 18 weeks of gestation. The
sequence of formation is largely ventral to dorsal, beginning
with the genu and progressing through the anterior body,
posterior body, splenium, and rostrum.
SWISS CHEESE
 Periventricular leukomalacia,
end-stage

In premature infants, the periventricular white matter is most

susceptible to hypoxic injury because it is supplied by
ventriculopetal penetrating arteries from the cortical
surface.End-stage PVL is characterized by cystic
encephalomalacia (Swiss cheese brain) with severe white
matter loss and ventriculomegaly.
POINT, WEDGE
 Dysembryoplastic
neuroepithelial tumor

Dysembryoplastic neuroepithelial tumors (DNETs) are

benign, slow-growing neuroepithelial tumors arising
from cortical or deep gray matter. Patients are typically
male and under age 20. The temporal lobe is the most
common location, followed by the frontal lobe.
PLASTIC, TOOTHPASTE
 Ependymoma

Ependymomas are benign tumors that arise

from ependymal cells lining the ventricles
and central canal of the spinal cord.
PEG, TAIL
 Chiari malformation

Chiari malformations involve a spectrum of complex

congenital anomalies, characterized by posterior fossa
hypoplasia and descent of the cerebellar tonsils over 3
to 5 mm below the foramen magnum.
PARALLEL VENTRICLES,
RACING CAR
Callosal agenesis
OPEN LIP
Open-lip schizencephaly
NODULAR, PEARLS ON A
STRING
Tuberous sclerosis
MUSHROOM
 Ulegyria

Ulegyria refers to cortical injury with preferential

atrophy of the deep cortical layers (cortical
laminar necrosis). The usual etiology is mild
hypoxic-ischemic injury in fullterm infants,
preferentially affecting the parasagittal watershed
areas.
MOLAR TOOTH
 Joubert syndromerelated
disorders

Joubert syndromerelated disorders (JSRD), or

cerebello-oculo-renal disor ders, are autosomal
recessive disruptions of midbrain-hindbrain
development with associated ocular, facial, hepatic,
renal, and digital anomalies.
LINEAR, RADIAL BANDS
 Tuberous sclerosis
LEOPARD SKIN, STRIPE,
TIGROID
 Metachromatic
leukodystrophy
KEYHOLE
 Hippocampal hypoplasia

Hippocampal hypoplasia can occur with a variety of

congenital brain malformations including callosal
agenesis, lissencephaly, schizencephaly, polymicrogyria,
holoprosencephaly, and heterotopia.
INTERHEMISPHERIC CYST
 Callosal dysgenesis
HOURGLASS, SHARK
TOOTH
Chiari II malformation
GAPING/HEART/WIDE
INCISURA
Chiari II malformation
FUSED THALAMI
Holoprosencephaly
FLAT FLOOR OF FOURTH
VENTRICLE
 Brainstem glioma
FIGURE EIGHT,
MENISCUS
 Tonsillar herniation
 FIGURE EIGHT,
HOURGLASS, OVAL
Lissencephaly, type I
 FENESTRATED FALX,
INTERDIGITATING/INTER
LOCKING GYRI
Chiari II malformation
DOUBLE CORTEX, THREE
LAYER CAKE
Band heterotopia
DIMPLE, NIPPLE
Schizencephaly
DIAMOND, STAR
Chiari II malformation
DIAMOND, KEYHOLE,
TRAPEZOID
 Dandy-Walker complex
DARK CEREBELLUM
Cerebellar infarcts
CYST WITH NODULE,
MURAL NODULE
 Juvenile pilocytic
astrocytoma
CORTICAL/SUBCORTICA
L ISLANDS, TUBERS
Tuberous sclerosis
 CONVOLUTED,
CURVILINEAR,
GYRIFORM, SERPENTINE,
TRAM LINE/TRACK
 Sturge-Weber syndrome
CLOSED LIP
Closed-lip schizencephaly
CLEFT-DIMPLE
Cortical dysgenesis
CAULIFLOWER
Choroid plexus tumor
 CANDLE
GUTTERING/WAX
 Tuberous sclerosis
BUTTERFLY
Adrenoleukodystrophy
BUMPY, COBBLESTONE,
NODULAR, PEBBLE,
VERRUCOUS
Lissencephaly, type II
BULLET/HEART/TOWERI
NG CEREBELLUM
Chiari II malformation
BUBBLY, FEATHERY, SOAP
BUBBLE, SWISS CHEESE
 Dysembryoplastic
neuroepithelial tumour
 BRIGHT SPOTS,
UNIDENTIFIED BRIGHT
OBJECTS
Neurofibromatosis type I
 BRIGHT RIM,
HYPERINTENSE RING
 Dysembryoplastic
neuroepithelial tumour
BOXCAR VENTRICLES
 Septo-optic dysplasia
BULL/RAM/STEER HORN,
Callosal agenesis
HORSESHOE,
ANGEL WING, TRIPLE
PEAK
Chiari II malformation
 ANGULAR, FAN,
SCALLOPED, TRIANGLE
Periventricular leukomalacia