Insights into the pathogenesis of cerebral lesions in incontinentia pigmenti

Hennel, Sabine J; Ekert, Paul G; Volpe, Joseph J; Inder, Terrie E

doi:10.1016/S0887-8994(03)00150-4

« Previous Next »Pediatric Neurology
Volume 29, Issue 2 , Pages 148-150, August 2003

Insights into the pathogenesis of cerebral lesions in incontinentia pigmenti

Sabine J Hennel, MD
- Department of Pediatrics, Murdoch Children’s Research Institute and Howard Florey Institute, and Royal Women’s and Royal Children’s Hospital, Melbourne, Australia
Paul G Ekert, MD, PhD
- Department of Pediatrics, Murdoch Children’s Research Institute and Howard Florey Institute, and Royal Women’s and Royal Children’s Hospital, Melbourne, Australia
Joseph J Volpe, MD
- Department of Neurology, Children’s Hospital and Harvard Medical School, Boston, Massachuasetts, USA
Terrie E Inder, MD
- Department of Pediatrics, Murdoch Children’s Research Institute and Howard Florey Institute, and Royal Women’s and Royal Children’s Hospital, Melbourne, Australia
- Department of Neurology, Children’s Hospital and Harvard Medical School, Boston, Massachuasetts, USA
- Communications should be addressed to: Dr. Inder; Murdoch Children’s Research Institute and Howard Florey Institute; Royal Children’s Hospital; Flemington Rd; Parkville VIC 3052, Australia.

Received 24 October 2002; accepted 17 February 2003.

Abstract

We report the case of a neonate with incontinentia pigmenti and seizures on day 4 of life who underwent magnetic resonance imaging and angiography scanning at 8, 13, and 21 days of age. The serial magnetic resonance images demonstrated the evolution of acute microvascular hemorrhagic infarcts in the periventricular white matter in the first week of life. The associated magnetic resonance angiogram findings consisted of decreased branching and poor filling of intracerebral vessels. This report adds important insights into the nature and timing of cerebral lesions in incontinentia pigmenti.