Hosting 728 files, 2951 images, and 4 contributors.

Random Case

Gatric Rupture
Joseph Junewick, MD FACR
over 9 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

Lissencephaly with heterotopia

Case Detail

Anatomy: Brain-Spine
Heather Borders, MD
Diagnostic Category: Developmental or Congenital
Created: over 10 years ago
Updated: over 10 years ago
Tags: PEDS
Modality/Study Types: MRI
Adobe32 PDF Imagej32 ImageJA


History of worsening seizures. Known diagnosis of Miller Dieker syndrome. No prior imaging.

Case Images


Lissencephaly with nodular subependymal heterotopia


Smooth brain with absence of gyral and sulcal development. Diminished white matter with an outer cortical layer separated from the inner thicker cortical layer by a thin cell sparse zone. Foci of CSF intensity in the cell sparse zone and periventricular white matter may represnt dysplastic myelination. Multiple foci of nodular subependymal heterotopia are also present in this case.


Neuroglial cells migrate along radial glial fibers with the help of various ligand interactions and cell adhesions. Various genetic mutations that affect this process will result in a spectrum of abnormal migration. A large deletion on 17p13.3 is seen with Miller Dieker.

Patients with classical lissencephaly, as in this case, are generally born with hypotonia. In patients with incomplete lissencephaly, clinical findings are usually less severe. Some patients with lissencephaly may have agenesis of the corpus callosum or cerebellar hypoplasia and these abnormalities should be looked for.

Miller Dieker syndrome is associated with type I lissencephaly as in this case. The patients will have characteristic facial features.


Barkovich fourth edition pediatric neuroimaging, pp. 346-
Statdx, Lissencephaly by Susan Blaser 6-2-2008.

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

Spectrum Health Helen Devos Childrens Hospital GE HealthCare