Destructive encephalopathy in incontinentia pigmenti: A primary disorder?

Avinoam Shuper, R. Nick Bryan, Harvey S. Singer

Research output: Contribution to journalArticlepeer-review


A 3-day-old infant with incontinentia pigmenti presented with acute encephalopathy associated with neuroradiographic findings of hemorrhagic necrosis and brain edema. No specific infectious, inflammatory, vascular, or metabolic abnormality was identified. We speculate that there is a variably expressed mutant protein in incontinentia pigmenti that could cause either developmental brain malformations or a destructive process.

Original languageEnglish
Pages (from-to)137-140
Number of pages4
JournalPediatric Neurology
Issue number2
StatePublished - 1990
Externally publishedYes


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