Hosting 728 files, 2951 images, and 4 contributors.

Random Case

Congenital C2 Spondylolysis
Joseph Junewick, MD FACR
over 10 years ago
Enter A Workflow
Standard2   Academic2

Please choose a workflow. A standard workflow allows you to browse the repository with full case detail; the academic workflow allows you to browse the repository with limited case detail revealed. Double click on the images to launch image viewer.

ARSt Case Repository

Classical Lissencephaly

Case Detail

Anatomy: Brain-Spine
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 8 years ago
Updated: over 8 years ago
Tags: PEDS
Modality/Study Types: MR
Adobe32 PDF Imagej32 ImageJA


Newborn male with seizures.

Case Images


Classical Lissencephaly


MR – Axial T2 images show a thin superficial neuronal layer separated from a thick deep neuronal layer by a hyperintense cell-sparse layer. Subependymal nodular heterotopias represent the extreme form of neuronal dysjuction (migratory arrest).


The germinal matrix lines the ventricular margins and gives rise to neuroblasts. Around 8 weeks gestation, neuroblasts begin to proliferate and associate with specialized bipolar glial cells. The glial cells extend from the ventricular margin to the pial surface and provide a scaffolding to allow peripheral migration of neuroblasts. Newly formed cells migrate outward through pre-existing cell layers to reach superficial cortex. Neuronal migration in the cortex results in 6 cell layers and is completed by 6–8-months gestation.

Lissencephaly is a devastating brain malformation associated with seizures, profound developmental delay, and often death. Lissencephaly associated with facial dysmorphism is referred to as Miller-Dieker syndrome and is associated with deletions of chromosome 17p13.3 (LIS1 gene). The LIS1 gene is responsible for functional dynein and normal formation of the glial scaffolding.

The lissencephalic brain has total or near complete absence of sulcation. The lack of cerebral convolution results in a vertical sylvian fissure and a “figure of eight” appearance of the cerebral hemispheres. Histologically, the cortex has four instead of the normal six cell layer cortex. Counterintuitively, the two most superficial layers of cortex are formed early by the neurons that migrated normally, whereas the deeper layers are composed of neurons formed later that did not migrate. The third layer contains astrocytes, oligodendroglial cells, and dysplastic neurons. The fourth layer is the densely cellular and radially oriented.


Pilz D, Stoodley N, Golden JA. Neuronal migration, cerebral cortical development, and cerebral cortical anomalies. J Neuropathol Exp Neurol (2002); 61:1–11.

Corporate Office: 616.363.7272, 3264 North Evergreen Drive, Grand Rapids, MI 49525

Spectrum Health Helen Devos Childrens Hospital GE HealthCare