Get a Group Membership for your Organization. Free Trial
Pricing
Free TrialLogin

Lissencephaly with band type Heterotopia – Severe

HIDE
PrevNext

0:00

This is an MRI of the brain in a child with

0:03

seizures and developmental delay,

0:05

and we can see a severe paucity of sulcation.

0:08

The brain surface is relatively featureless.

0:11

We see a very rudimentary Sylvian fissure here

0:15

on the right and a shallow version of a

0:19

similar structure here on the left.

0:21

Otherwise,

0:23

very featureless, we see a little bit of,

0:26

in this parasagittal aspect of the frontal lobes,

0:29

we see the anterior aspect of the cingulate gyrus.

0:31

If we go here to the sagittal image,

0:34

we see the parieto-occipital fissure,

0:36

we see the calcarine sulcus,

0:38

but otherwise, we're seeing very few sulcations

0:42

within the cuneus of the occipital lobe,

0:44

as well as the lingual gyrus of the occipital lobe.

0:47

If we go a little bit off to the side,

0:48

we're also seeing minimal sulcation,

0:51

very featureless brain.

0:53

In addition to the surface findings of

0:56

a relatively featureless brain,

0:58

we're seeing a very thickened,

0:59

what looks like cortex,

1:01

but not all of this is really cortex.

1:04

If we look at an image here,

1:08

we can see the gray matter at the periphery,

1:13

but all of this is actually an intervening area.

1:17

Here in the right occipital lobe,

1:19

we can see this is the cortex.

1:20

These little areas where it looks slightly bright

1:24

is actually white matter.

1:26

It is what's called the cell sparse zone.

1:29

Then beneath that is an additional

1:32

band of gray matter.

1:33

That band of gray matter follows the surface of

1:36

the brain throughout both cerebral hemispheres.

1:38

This deeper gray matter that's in a band like

1:41

configuration is not in the normal location,

1:45

so it's called heterotopic gray matter.

1:47

This pattern of heterotopic gray matter

1:50

is called band heterotopia.

1:51

The smooth surface of the brain is known

1:54

as lissencephaly.

1:56

Lissencephaly,

1:57

especially the more severe forms,

1:59

is often associated with a

2:00

band type heterotopia.

2:02

Band heterotopia is an abnormality of neuronal migration.

2:06

We can see other abnormalities here,

2:08

that there's a severe loss of the cerebral

2:11

white matter volume.

2:13

Overall,

2:13

the brain doesn't have a lot of the

2:15

landmarks we're used to seeing.

2:16

The lateral ventricles have a slightly

2:18

dysmorphic appearance due to the abnormal

2:21

configuration of the surrounding white matter.

2:23

And we can see the cerebellum looks closer

2:27

to normal than the cerebral hemispheres.

2:30

The cerebellar volume is a little

2:32

bit lower than usually seen,

2:34

but compared to the cerebral hemispheres,

2:36

they're relatively preserved.

2:38

And this is an abnormality known as

2:40

lissencephaly with bandtype heterotopia.

Report

Description

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Tags

Pediatrics

Neuroradiology

MRI

Congenital

Brain

© 2024 MRI Online. All Rights Reserved.

Contact UsTerms of UsePrivacy Policy