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Right epidermal nevus syndrome and hemifacial lipomatosis (MRI)

(A, B) MRI of axial and coronal views of the brain, showing right hemimegalencephaly and islands of heterotopic gray matter within deep subcortical white matter of centrum semiovale. (C) Coronal MRI shows an expansion of the right diploic space between the inner and outer tables of the cranial bones overlying the dysplastic cerebral hemisphere. The lateral ventricles show straightening of the right frontal horn, a characteristic sign of hemimegalencephaly. (Contributed by Dr. Laura Flores-Sarnat.)

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Associated Disorders

  • Cardiac anomalies
  • Hemimegalencephaly
  • Infantile spasms
  • Ocular anomalies
  • Vitamin D-resistant rickets