Yervant Erdogan - Double Cortex Syndrome.

"Double cortex syndrome, also known as subcortical band heterotopia, is a congenital brain abnormality primarily affecting females that results from aberrant migration of neurons during development of the cortex. The name double cortex comes from the appearance of an extra layer of neurons (nerve cells) that are under the normal gray matter of brain cortex. MRI shows a continuous band of heterotopic gray matter located between the cortex and ventricular walls, separated from them by a thin layer of white matter. The thickness of the band correlates with the ultimate neurologic outcome. The symptoms people with subcortical band heterotopia experience can vary from severe intellectual disability and epilepsy to normal intelligence with mild or no epilepsy. Focal seizures are seen in about 50% of individuals and generalized seizures in about 50%. Behavior problems may also be observed. Double cortex syndrome is most often caused by mutations in the DCX gene. The condition is inherited in an X-linked dominant pattern.  Some cases may be caused by a small deletion on chromosome 17 involving the LIS1 gene. Management consists of seizure control. In about 65% the epilepsy is refractory to antiepileptic therapy." -www.diseaseinfosearch.org


Regards,
Yervant Erdogan.