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Diseases

Genetic and Rare Diseases Information Center (GARD)

Hemimegalencephaly


Other Names for this Disease
  • Macrencephaly
  • Unilateral Megalencephaly
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

Hemimegalencephaly is a rare malformation involving one side of the brain.[1][2] It may occur alone or in association with other syndromes such as Proteus syndrome, tuberous sclerosis, linear sebaceous nevus syndrome, neurofibromatosis, Sturge-Weber syndrome, or Klippel-Trenaunay syndrome.[1] Children with this disorder may have a large, asymmetrical head accompanied by seizures, partial paralysis, and impaired cognitive development.[2][3] Because the seizures associated with hemimegalencephaly are difficult to treat with anticonvulsant medications, a surgery called hemispherectomy is often the most successful treatment.[1][3] The cause of hemimegalencephaly is not fully understood, but involves a disturbance of cells early in development and likely involves genes involved in patterning and symmetry.[1][2]

Last updated: 11/27/2015

References

  1. Crino P. Hemimegalencephaly. National Organization for Rare Disorders (NORD). 2012; http://rarediseases.org/rare-diseases/hemimegalencephaly/. Accessed 11/27/2015.
  2. NINDS Megalencephaly Information Page. National Institute of Neurological Disorders and Stroke (NINDS). June 30, 2015; http://www.ninds.nih.gov/disorders/megalencephaly/megalencephaly.htm. Accessed 11/27/2015.
  3. Hemimegalencephaly. Johns Hopkins Medicine. http://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/epilepsy/seizures/causes/hemimegalencephaly.html. Accessed 11/27/2015.
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Basic Information

  • MedlinePlus provides more information on head and brain malformations. MedlinePlus is a Web site designed by the National Library of Medicine to help you research your health questions.
  • The Merck Manuals Online Medical Library has an information page on brain anomalies in general. Click on the link above to view the information page.
  • The Cortical Foundation has an information page for hemimegalencephaly. Click on the link above to access this information page.
  • Information on cephalic disorders and megalencephaly can be found on the National Institute of Neurological Disorders and Stroke (NINDS) Web site. To view the information pages click on the links above.
  • The Johns Hopkins Medicine Web site provides information on hemimegalencephaly. Click on the link above to view this information page.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • 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 Hemimegalencephaly. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Macrencephaly
  • Unilateral Megalencephaly
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.