Subcortical grey matter band heterotopia

Case contributed by Mohammad A. ElBeialy


Mild epilepsy and intellectual disability.

Patient Data

Age: 6 years.
Gender: Female

Grey matter band heterotopia or double cortex syndrome with a band of grey matter located deeply and almost parallel to the cortex with similar signal intensity; the double cortical layers are separated by a thin white matter layer. The overlying cortex is normal with no pacchygyria, polymicrogyria or lissencephaly. The ventricles are slightly prominent in size for the patient's age.

No evidence of acute intracranial bleeding, recent infarct, significant focal mass effect, or hydrocephalus is seen. The basal cisterns are patent.  The signal intensity of the cerebellum, and of the brainstem is within normal limits.. No abnormal extra-axial fluid collection is visible. Flow voids of the major intracranial vessels appear grossly preserved.  The paranasal sinuses and mastoid air cells are clear. No gross orbital mass.    

Case 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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