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Midline epidermal nevus and intractable tonic spasms in an infant (video-EEG)

(A) A 1-month-old, term-infant girl with intractable tonic spasms since the second week of life and a midline epidermal nevus. CT and MR imaging studies demonstrated left hemimegalencephaly. The EEG shows a hemisuppression-burst pattern of paroxysmal activity. (B) Tonic seizures alternate with infantile spasms. The asymmetrical hemisuppression-burst pattern in the left hemisphere appeared semiperiodically during both waking and sleeping states. (Contributed by Dr. Laura Flores-Sarnat.)

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Associated Disorders

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