Subcortical band heterotopia
Other Names for this Disease
- Double cortex syndrome
- Double cortex
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Subcortical band heterotopia, also known as double cortex syndrome, is a condition of abnormal brain development that is present from birth. This condition which primarily affects females, occurs when neurons migrate to an area of the brain where they are not supposed to be (heterotopia), and form abnormal areas that appear as band-like clusters of white tissue underneath the gray tissue of the cerebral cortex (subcortical), creating the appearance of a double cortex. Symptoms associated with subcortical band heterotopia vary from severe intellectual disability and epilepsy to normal intelligence with mild or no epilepsy. Subcortical band heterotopia 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.
Last updated: 12/3/2012
- Hehr U, Uyanik G, Aigner L, Couillard-Despres S, Winkler J. DCX-Related Disorders. GeneReviews. 2011; http://www.ncbi.nlm.nih.gov/books/NBK1185/. Accessed 12/3/2012.
- DCX. Genetics Home Reference (GHR). 2008; http://ghr.nlm.nih.gov/gene=dcx. Accessed 12/3/2012.
- Passaro EA. Neuroimaging in Epilespy Surgery. Medscape Reference. 2011; http://emedicine.medscape.com/article/1155295-overview. Accessed 12/3/2012.
- Genetics Home Reference (GHR) contains information on Subcortical band heterotopia. This website is maintained by the National Library of Medicine.
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