Band heterotopia (x-linked)

Case contributed by A.Prof Frank Gaillard

Presentation

Absence / atonic seizures.

Patient Data

Age: 30 years
Gender: Female

MRI brain

Modality: MRI

Extensive bilateral predominantly frontal and to a lesser degree parietal subcortical thin grey matter bands are seen, with T1 and T2 signal isointense to grey matter. They are separated from the overlying cortex by white matter. Sulcation is normal. 

Case Discussion

This case illustrates extensive, predominantly frontal, subcortical band heterotopia in a patient with established x-linked band heterotopia syndrome - presumably DCX gene located on the long arm of chromosome X (the predominantly frontal distribution is also in favour of this). This condition represents the less severe end of the Lissencephaly type I - subcortical band heterotopia spectrum of malformations. 

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Case Information

rID: 15814
Case created: 13th Nov 2011
Last edited: 6th Nov 2015
Inclusion in quiz mode: Included

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