Double cortex syndrome

Case contributed by Frank Gaillard
Diagnosis certain


Intractable seizure and developmental delay.

Patient Data

Age: 8
Gender: Female

MRI brain


MRI of an 8 year old demonstrates a continuous ribbon of grey matter seen just deep to the cortex, separated from it with a thin layer of white matter. The overlying brain demonstrates some shallowness of sulci. Incidental cavum septum pellucidum et vergae is noted. 

Case Discussion

This case illustrates typical and obvious features of double cortex (aka band heterotopia) without obvious lissencephaly. This is likely to be x-linked (female patient) due to mutations of the DXC gene located on the long arm of chromosome X. 

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