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Focal Cortical Dysplasia
Focal Cortical Dysplasia
PROLIFERATION/ ORGANIZATION/
MIGRATION
APOPTOSIS MATURATION
• *
Cerebral Cortex
• Normal neocortex
– 6-layered neocortex
present in over 90% brain
• Olfactory paleocortex &
hippocampal archicortex
*
composed of 3 layers
• Normal neocortex is 1-3 *
mm in thickness
Magnified T1-weighted image
– Thinner at depth of demonstrating normal gyri.
sulcus (*) indicates depth of sulcus,
(*)
green arrow indicates crown of
– Thicker at crown of gyrus
gyrus.
(green arrow)
Malformations of Cortical Development
(MCD)
MCDs are microro-or macroscopic abnormalities of the
cerebral cortex that result from interruption of
normal cortical formation
• This presentation will review the • Most patients with MCDs have
imaging findings of the following MCDs: epilepsy
– Microcephaly with Simplified Gyral – MCDs cause 25-40% of intractable,
medically refractory epilepsy
Pattern (MSG)
– Exceptions: MSG
– Classic
Lissencephaly
(agyria-
pachygyria)
– Band Heterotopia
– Gray Matter (GM)
Heterotopia
– Polymicrogyria
(PMG)
Malformations of Cortical
Development (MCDs)
• MCDs have been increasingly recognized
in recent
decades due to improved MRI resolution
• At least 75% of patients with MCDs have epilepsy
PROLIFERATION/ ORGANIZATION/
MIGRATION
APOPTOSIS MATURATION
Band Heterotopia
Type IIb Focal Cortical
Dysplasia
Gray Matter (Nodular)
Heterotopia
Hemimegalencephaly
Schizencephaly
Polymicrogyria
• FCDs are the most important etiology of focal, medically refractory epilepsy
– found in almost ½ of pathologic specimens from surgery done for
refractory epilepsy
• Type IIb FCD is considered a disorder of proliferation
9-year-old with drug-resistant epilepsy. Axial T2- Note additional findings of scattered cortical
weighted image shows a large wedge-shaped thickening, GW junction blurring (→), as well
area of T2-hyperintensity involving both GM & as ill-defined extension and tapering into the
WM in the right parietal lobe. Margins of the WM along the anterior margin of the lesion (*)
abnormality are indicated by (→). on this image.
Disorders of Proliferation
Focal Cortical Dysplasia, Type IIb
Axial PET image shows focal hypometabolism in the right anterior frontal lobe (→).
This triggered reevaluation of the patient’s prior MRI (12-year-old with intractable
epilepsy). Upon additional review, a funnel shaped area of high FLAIR signal was found
in the same region (→), compatible with a subtle cortical dysplasia (“bottom of the
sulcus sign”). The transmantle extent is best seen on sagittal FLAIR images (→).
Symptom
• Focal cortical dysplasia may involve any part of the brain, may vary in size and
location and may be multifocal
• Epilepsy, sometimes associated with mental retardation, particularly with early seizure onset.
• There are no significant neurological deficits despite large areas of brain tissue occupied by a lesion.
• Symptoms appear at any age, mostly in childhood, but also occur in adults. Epilepsy is usually drug-
resistant.
• Patients with FCD type II manifest earlier onset comparing to type I
• Focal cortical dysplasia type I is related to temporal lobe seizures .
• In patients with FCD type II, multilobar lesions are found, involving hemisphere, often with
extratemporal location and mainly in the frontal lobe. Therefore seizures with early onset in neonatal
period or childhood are more likely FCD type II with multilobar or hemispheric lesion, while FCD
type I with a small focus, usually in the temporal lobe, predominates in adults
Global developmental delay (GDD) atau keterlambatan
perkembangan global (KPG)
• Definisi : Merupakan keterlambatan bermakna pada lebih dari dua domain
perkembangan.
Tanuwijaya S. Kebutuhan dasar tumbuh kembang anak. Dalam: Narendra M, Sularyo S, Soetjiningsih, Suyitno H, Ranuh IGN, penyunting. Buku ajar tumbuh kembang
anak dan remaja, edisi ke-1. Jakarta: Sagung Seto; 2002.h.13-21.
https://www.cps.ca/en/documents/position/evaluation-of-the-child-with-global-developmental-delay-and-
Table 1. Causes of global developmental delay/intellectual disability
intellectual-disability
Broad category Possible causes Proportion of diagnostic yield*
Prenatal history
Prenatal ultrasound
Screening for fetal aneuploidy
Maternal diabetes or hypertension
Infections
Exposure to medications or toxins
Birth history
Weight and height
Head circumference
Apgar score
Length of hospitalization
•
References & Suggested Readings
• Raybaud C, Widjaja E. Development & Dysgenesis of the Cerebral
Cortex: Malformations of Cortical Development. Neuroimaging Clin
N Am. 2011: 483-543
• Leach JL, Greiner HM, Lilles L, Mangano FT. Imaging Spectrum of
Cortical Dysplasias in Children. Semin Roentgenol. 2014: 99-111