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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.

Treatment

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How might hemimegalencephaly be treated?

Because the seizures associated with hemimegalencephaly are difficult to control with anti-epileptic medications, most patients undergo surgery to separate one hemisphere of the brain from the other (hemispherectomy). There are several surgical options to consider. One is functional hemispherectomy which involves severing the nerves and tissue connecting one side of the brain to the other, leaving the brain within the skull. Another is a complete or anatomic hemispherectomy in which the affected side of the brain is surgically removed. These surgeries are usually performed by a neurosurgeon with experience treating epilepsy syndromes.[1][2] Over time, the remaining side of the brain may take over the functions lost.[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. Hemimegalencephaly. Johns Hopkins Medicine. http://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/epilepsy/seizures/causes/hemimegalencephaly.html. Accessed 11/27/2015.


GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

    Finding Treatment Information

Clinical Trials & Research for this Disease

  • The Christopher A. Walsh Laboratory is interested in the development of the cerebral cortex. Abnormal development of the cerebral cortex in humans results in epilepsy, autism, mental retardation, dyslexia, and other learning disorders, and perhaps some psychiatric conditions as well. Several of their projects are directed at trying to understand the basic biology of the cortex by studying the mutations that disturb its development. Conditions that they are currently researching include: double cortex syndrome, periventricular heterotopia, schizencephaly, perisylvian polymicrogyria and Walker Warburg syndrome. Click on the link above to access further information about this research.

  • The Cortical Foundation provides information on participating in research for cortical malformations of the brain.
  • Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition. 
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.