Double Cortex Syndrome

Subcortical band heterotopia

Overview

Type of disease: Rare conditions

Double cortex syndrome, also known as Subcortical band heterotopia, is a rare genetic disorder that involves problems with the development of the brain. In this condition specific brain cells called neurons, travel to areas of the brain where they aren’t supposed to be and clump together. Individuals with double cortex syndrome may have severe intellectual disability, seizures, and behavioral problems. The severity of these symptoms varies among individuals; some individuals have normal mental development and no seizures. The condition is more common in females than in males.

There are two genes that are linked to double cortex syndrome. A few cases have been linked to a change (mutation) in the LIS1 gene on the 17th chromosome and the majority of cases are caused by a change (mutation) in the DCX gene on the X-chromosome.
When the condition is caused by a mutation in the DCX gene it is passed through families in an X-linked dominant manner, which means that only one mutation is needed in order to have this condition. For more information see X-linked dominant inheritance.

There is no cure for double cortex syndrome, but there are treatments available to help manage seizures. If you or a family member has been diagnosed with double cortex syndrome, talk with your doctor about the most current treatment options. Support groups are also good resources of support and information.

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