High-dose glucocorticoid therapy in the management of seizures in neonatal incontinentia pigmenti: A case report

David S. Wolf, W. Christopher Golden, Julie Hoover-Fong, Carolyn Applegate, Bernard A. Cohen, Emily L. Germain-Lee, Morton F. Goldberg, Thomas O. Crawford, Estelle B. Gauda

Research output: Contribution to journalArticlepeer-review

Abstract

Incontinentia pigmenti is an X-linked dominant disorder resulting from a mutation of IKBKG. This disorder has a classic dermatologic presentation, but neurologic involvement, with seizures and cortical infarction, can arise shortly after birth. There are no specific therapies available for the manifestations of incontinentia pigmenti. Here, we describe the clinical, electrographic, and neuroradiologic effect of systemic glucocorticoid therapy in a neonate with incontinentia pigmenti manifesting an epileptic encephalopathy. Treatment with dexamethasone led to a dramatic reduction in seizure activity and improvement in bullous lesions. A novel mutation in IKBKG is also reported.

Original languageEnglish (US)
Pages (from-to)100-106
Number of pages7
JournalJournal of child neurology
Volume30
Issue number1
DOIs
StatePublished - Jan 2014

Keywords

  • Incontinentia pigmenti
  • Ischemic encephalopathy
  • Neonatal seizures

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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