Neuroimaging features of epidermal nevus syndrome

AJNR Am J Neuroradiol. 2003 Aug;24(7):1468-70.

Abstract

Epidermal nevus syndrome is a kind of neurocutaneous syndrome that is associated with epidermal nevus and a variety of congenital CNS disorders. Clinical presentations include seizures, paresis, mental retardation, and developmental delay. We report three cases with MR imaging and magnetoencephalography findings; one patient underwent ictal and interictal single photon emission CT. Both structural and functional imaging studies indicated that the frontal lobes had lesser involvement or were intact. One patient underwent hemispherectomy because of the medically intractable seizure. He remained seizure free with topiramate monotherapy.

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Central Nervous System / abnormalities*
  • Central Nervous System / diagnostic imaging
  • Child, Preschool
  • Epidermis / abnormalities*
  • Epidermis / diagnostic imaging
  • Female
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Magnetoencephalography
  • Male
  • Neurocutaneous Syndromes / congenital
  • Neurocutaneous Syndromes / diagnosis*
  • Radiography
  • Seizures / congenital
  • Seizures / diagnosis
  • Tomography, Emission-Computed, Single-Photon