Incontinentia Pigmenti With Neonatal Encephalopathy: A Case Report and Literature Review

gene. Although corticosteroid therapy was not initiated immediately, it was later introduced during infancy in response to electrographic hypsarrhythmia, resulting in successful resolution. Additionally, we conducted a retrospective review of the timing of seizure onset and the use of systemic corticosteroids as reported in the literature.ConclusionEarly clinical evaluation for central nervous system involvement is crucial in patients with IP. Given the genetic predisposition to neonatal encephalopathy and acute symptomatic seizures, symptomatic management with antiseizure medications is often required. Although no randomized clinical trials have been conducted, early immunotherapy with corticosteroids may be a viable option for managing acute neurologic complications. This report aims to promote early recognition of IP-associated neonatal encephalopathy, support timely intervention, and inform long-term follow-up strategies.

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Publication Details

Journal
Journal of Child Neurology
Published
2026-03-31
DOI
https://doi.org/10.1177/08830738261415990
Primary Topic
Genetic and rare skin diseases.
Type
article
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article

Incontinentia Pigmenti With Neonatal Encephalopathy: A Case Report and Literature Review

Chrysanthy Ikonomidou
Journal of Child Neurology
Genetic and rare skin diseases.
article

Incontinentia Pigmenti With Neonatal Encephalopathy: A Case Report and Literature Review

Chrysanthy Ikonomidou
article en

Abstract

gene. Although corticosteroid therapy was not initiated immediately, it was later introduced during infancy in response to electrographic hypsarrhythmia, resulting in successful resolution. Additionally, we conducted a retrospective review of the timing of seizure onset and the use of systemic corticosteroids as reported in the literature.ConclusionEarly clinical evaluation for central nervous system involvement is crucial in patients with IP. Given the genetic predisposition to neonatal encephalopathy and acute symptomatic seizures, symptomatic management with antiseizure medications is often required. Although no randomized clinical trials have been conducted, early immunotherapy with corticosteroids may be a viable option for managing acute neurologic complications. This report aims to promote early recognition of IP-associated neonatal encephalopathy, support timely intervention, and inform long-term follow-up strategies.

Journal of Child Neurology
University of Wisconsin–Madison (US)
Good health and well-being
Openalex Percentile: Top 30%
Genetic and rare skin diseases.
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