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This is an MRI of the brain in a one-week-old

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child that had an in utero abnormality

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that wanted to be further characterized.

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One of the first things we see is this very,

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very featureless surface sulcation

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pattern to the brain.

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We're seeing a rudimentary Sylvian

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fissure on both sides.

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At most,

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it looks sort of like a large figure eight,

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but we're not seeing any other major sulci.

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Within that,

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we're seeing enlargement of

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both lateral ventricles,

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more pronounced posteriorly.

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On the brain,

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we're actually seeing something that we saw on

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the other cases.

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We saw the peripheral cortex.

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We see a cell sparse zone that's bright

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on T2-weighted imaging.

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And then, we see what is a band of gray matter

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like substance called gray matter heterotopia.

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So, this is a very severe case of lissencephaly

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with band-type heterotopia.

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At this point,

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instead of just lissencephaly,

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which means smooth brain,

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it can also be referred to as agyria because

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there's essentially no gyri.

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The coronal image here also shows.

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You can see the temporal lobe

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is just one single curve on the periphery,

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with no surface undulations.

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We see that subtle curvature for this concavity,

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for the Sylvian fissure,

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and we're not seeing any sulcation

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whatsoever in the frontal lobes.

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This is a fairly severe appearance.

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The patient beforehand had a head ultrasound

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which also showed a very featureless

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temporal lobe,

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very featureless parasagittal cortex here

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in the frontal lobes.

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The one thing to be aware of, though,

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is to be careful on ultrasound,

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especially to not overcall lissencephaly

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in the setting of severe prematurity.

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The sulcation pattern that looks

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abnormal in a term infant

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could be normal at 32 weeks or 28 weeks.

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So in a premature infant,

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where some of the brain development

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has not yet taken place,

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we have to be careful not to overcall lissencephaly.

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That said,

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this severe abnormality is definitely

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a true lissencephaly.

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The MRI confirms the cell sparse zone

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and the band-type heterotopia.

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So this is a severe case of lissencephaly with

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band-type heterotopia with an agyric pattern.