Destructive encephalopathy in incontinentia pigmenti: A primary disorder?

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

Research output: Contribution to journalArticlepeer-review

40 Scopus citations


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 (US)
Pages (from-to)137-140
Number of pages4
JournalPediatric Neurology
Issue number2
StatePublished - 1990

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Developmental Neuroscience
  • Clinical Neurology


Dive into the research topics of 'Destructive encephalopathy in incontinentia pigmenti: A primary disorder?'. Together they form a unique fingerprint.

Cite this