Double Cortex Syndrome

Common Name(s)

Double Cortex Syndrome, Subcortical band heterotopia

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.  
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Double Cortex Syndrome" for support, advocacy or research.

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Double Cortex Syndrome" returned 1 free, full-text research articles on human participants. First 3 results:

Doublecortin, a brain-specific gene mutated in human X-linked lissencephaly and double cortex syndrome, encodes a putative signaling protein.
 

Author(s): J G Gleeson, K M Allen, J W Fox, E D Lamperti, S Berkovic, I Scheffer, E C Cooper, W B Dobyns, S R Minnerath, M E Ross, C A Walsh

Journal: Cell. 1998 Jan;92(1):63-72.

 

X-linked lissencephaly and "double cortex" are allelic human disorders mapping to Xq22.3-Xq23 associated with arrest of migrating cerebral cortical neurons. We identified a novel 10 kb brain-specific cDNA interrupted by a balanced translocation in an XLIS patient that encodes a novel ...

Last Updated: 16 Mar 1998

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The terms "Double Cortex Syndrome" returned 0 free, full-text review articles on human participants.

 
 
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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Analgesic Effect of Cathodal tDCS Over Right DLPFC in Subjects With Muscular TMD: a Double Blind Crossover RCT
 

Status: Not yet recruiting

Condition Summary: Craniomandibular Disorders

 

Last Updated: 28 May 2014

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Trial of RAD001 and Neurocognition in Tuberous Sclerosis Complex (TSC)
 

Status: Recruiting

Condition Summary: Tuberous Sclerosis Complex

 

Last Updated: 24 Jun 2014

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A Study of Everolimus in the Treatment of Neurocognitive Problems in Tuberous Sclerosis
 

Status: Recruiting

Condition Summary: Tuberous Sclerosis

 

Last Updated: 4 Oct 2013

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