Subcortical grey matter band heterotopia

Discussion:

Subcortical band heterotopia, also known as double cortex syndrome, is an X-linked dominant condition that affects almost exclusively females. Subcortical band heterotopia is most often caused by mutations in the DCX gene (some cases may be caused by a small deletion on chromosome 17 involving the LIS1 gene) which results in arrested migration of neurons with formation of band-like clusters of grey matter layer within the centrum semiovale or subcortical white matter underneath the cerebral cortex (subcortical), giving the characteristic appearance of a double cortex.

Band heterotopia is mostly linked to lissencephaly and always referred to lissencephaly type I - subcortical band heterotopia spectrum

Symptoms associated with subcortical band heterotopia are variable, from severe intractable to mild or even rarely no epilepsy, developmental delay or intellectual disability.

Management is always symptomatic for control of epilepsy. 

The images are courtesy of Dr. Shankar Bakde.

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