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Diseases

Genetic and Rare Diseases Information Center (GARD)

Bilateral frontal polymicrogyria


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Overview

Bilateral frontal polymicrogyria is one of the rarest subtypes of polymicrogyria. It is a symmetric and bilateral form (in both brain hemispheres) that only involves the frontal lobes without including the area located behind the Sylvius fissure or the area located behind the Rolando sulcus. Some researchers classify the condition into two different forms: bilateral frontal polymicrogyria and the bilateral frontoparietal. Signs and symptoms included delayed motor and language milestones; spastic (stiffness) hemiparesis (weakness in one side of the body) or quadriparesis (weakness in all four limbs of the body); and mild to moderate intellectual disability. Seizures may also be present. The frontoparietal form is caused by changes (mutations) in the GPR56 gene but the cause for the frontal form of polymicrogyira is still not known. Treatment is based on the signs and symptoms present in each person.[1][2][3]
Last updated: 10/11/2015

References

  1. Chang B, Walsh CA, Alpse K & Bodel A. Polymicrogyria Overview. Gene Review. August, 2007; http://www.ncbi.nlm.nih.gov/books/NBK1329/. Accessed 10/2/2015.
  2. Leventer R.J & cols. Clinical and imaging heterogeneity of polynicrogyria: a study of 328 patients. Brain. May, 2010; 133(5):1415-1427. http://brain.oxfordjournals.org/content/brain/early/2010/04/19/brain.awq078.full.pdf. Accessed 10/2/2015.
  3. Jansen A. & Andermann E. Genetics of the polymicrogyria syndromes. J Med Genet. 2005; 42:369–378. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1736054/pdf/v042p00369.pdf. Accessed 10/2/2015.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Bilateral frontal polymicrogyria. This website is maintained by the National Library of Medicine.

In Depth Information

  • 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.
  • 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 Bilateral frontal polymicrogyria. Click on the link to view a sample search on this topic.
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.