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.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

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 5 free, full-text research articles on human participants. First 3 results:

Sleep spindle activity in double cortex syndrome: a case report.
 

Author(s): Emilia Sforza, Jean-Pierre Marcoz, Giovanni Foletti

Journal: Epileptic Disord. 2010 Sep;12(3):228-31.

 

Cortical dysgenesis is increasingly recognised as a cause of epilepsy. We report a case with double cortex heterotopia and secondarily generalized seizures with a generalised spike wave pattern. During the course of the disease, the child developed electrical status epilepticus in ...

Last Updated: 8 Sep 2010

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Parkinsonism induced by VNS in a child with double-cortex syndrome.
 

Author(s): Arthur Cukiert, Pedro Paulo Mariani, Jose Augusto Burattini, Cristine Mella Cukiert, Cassio Forster, Carla Baise, Meire Argentoni-Baldochi, Valeria Mello

Journal: Epilepsia. 2009 Dec;50(12):2667-9.

 

We describe a child with epilepsy associated with double-cortex syndrome in whom vagus nerve stimulation (VNS) generated parkinsonian symptoms. A 13-year-old girl presented with refractory secondary generalized epilepsy from the age of 6 years and mental retardation. Her electroencephalography ...

Last Updated: 21 Dec 2009

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Surgical resection for intractable epilepsy in "double cortex" syndrome can yield adequate results.
 

Author(s): P C Tai, J D S McKean, B M Wheatley, D W Gross

Journal: Epilepsia. 2004 May;45(5):562-3; author reply 563-4.

 

Last Updated: 22 Apr 2004

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
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

Phase III Trial of Topical Formulation of Sirolimus to Skin Lesions in Patients With Tuberous Sclerosis Complex (TSC)
 

Status: Not yet recruiting

Condition Summary: Tuberous Sclerosis; Angiofibroma; Hypomelanotic Macule; Plaque

 

Last Updated: 21 Dec 2015

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A Randomized Controlled Trial of Cannabidiol (GWP42003-P, CBD) for Seizures in Tuberous Sclerosis Complex (GWPCARE6)
 

Status: Recruiting

Condition Summary: Tuberous Sclerosis Complex; Seizures

 

Last Updated: 11 Oct 2016

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Preventing Epilepsy Using Vigabatrin In Infants With Tuberous Sclerosis Complex
 

Status: Not yet recruiting

Condition Summary: Tuberous Sclerosis Complex

 

Last Updated: 28 Jul 2016

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