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Diseases

Genetic and Rare Diseases Information Center (GARD)

Subcortical band heterotopia


Other Names for this Disease
  • Double cortex syndrome
  • Double cortex
  • DC
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

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.[1][2] Subcortical band heterotopia is most often caused by mutations in the DCX gene. The condition is inherited in an X-linked dominant pattern.[1] Some cases may be caused by a small deletion on chromosome 17 involving the LIS1 gene.[1][3] Management consists of seizure control.[1] 
Last updated: 12/3/2012

References

  1. 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.
  2. DCX. Genetics Home Reference (GHR). 2008; http://ghr.nlm.nih.gov/gene=dcx. Accessed 12/3/2012.
  3. Passaro EA. Neuroimaging in Epilespy Surgery. Medscape Reference. 2011; http://emedicine.medscape.com/article/1155295-overview. Accessed 12/3/2012.
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Basic Information

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Subcortical band heterotopia. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Double cortex syndrome
  • Double cortex
  • DC
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.