What is the role of imaging studies in the workup of epidermal nevus syndrome (ENS)?

Updated: Mar 26, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Answer

MRIs can be used to evaluate intracranial involvement. MRIs may show cerebral atrophy, dilated ventricles, hemimegalencephaly, pachygyria, or enlarged white matter.

Multiple abnormalities are found on neuroimaging studies in patients with Jadassohn nevus phacomatosis. Findings include hemimegalencephaly (usually ipsilateral to the major skin lesions and contralateral to neurologic deficits), cortical atrophy, pachygyria, cortical heterotopias, agenesis of the corpus callosum, and Dandy-Walker syndrome.


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