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Radiology · Neuroimaging: neuronal migration disorder
Sample itemAn infant with developmental delay and seizures has an MRI showing a smooth brain surface with absent sulci and a thick cortex. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Lissencephaly
Lissencephaly, or smooth brain, is a neuronal migration disorder with absent gyri, thick cortex and shallow Sylvian fissures, presenting with seizures and delay. Holoprosencephaly shows failure of hemispheric separation, and hydranencephaly shows loss of hemispheres replaced by CSF. Dandy-Walker involves the posterior fossa.
Option by option
A. Lissencephaly (correct)
Correct. Absent sulci with a thick cortex is lissencephaly.
B. Hydranencephaly
Hydranencephaly shows a CSF-filled cranium with absent cortex.
C. Dandy-Walker malformation
Dandy-Walker malformation shows a cystic posterior fossa with an absent vermis.
D. Holoprosencephaly
Holoprosencephaly shows a single ventricle and fused hemispheres.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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