title: Cerebral Arteriopathy in a Newborn With Incontinentia Pigmenti.
abstract: BACKGROUND
  Incontinentia pigmenti is a rare X-linked neurocutaneous disorder that can present in the neonatal period with seizures and encephalopathy. Brain magnetic resonance imaging and magnetic resonance angiography may reveal cerebral infarction and arteriopathy.
  
  
  METHODS
  We describe a neonate with the typical rash of incontinentia pigmenti along with seizures and brain magnetic resonance imaging abnormalities.
  
  
  RESULTS
  Brain magnetic resonance imaging, magnetic resonance angiography, and magnetic resonance spectroscopy at age one week revealed chronic and acute brain injury, arteriopathy of the small and medium-sized cerebral vessels, and elevation of a lactate metabolite peak. By age six months, her magnetic resonance angiogram had normalized. At age 2.5 years, she has well-controlled complex partial seizures, global developmental delay, and residual hemiplegia.
  
  
  CONCLUSIONS
  Despite extensive cerebral arteriopathy in association with incontinentia pigmenti, this girl had a relatively stable early clinical course, steady developmental progress over time, and seizures that have been well controlled. Later brain imaging revealed resolution of the arteriopathy.
authors.affiliation: Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas. Electronic address: MulkeySarah@uams.edu.
authors.forename: Sarah B
authors.initials: SB
authors.lastname: Mulkey
authors.affiliation: Department of Radiology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Raghu H
authors.initials: RH
authors.lastname: Ramakrishnaiah
authors.affiliation: Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas.
authors.forename: Tonya M
authors.initials: TM
authors.lastname: Balmakund
mesh_terms: D001921:Brain / Q000098:blood supply / Q000254:growth & development / Q000378:metabolism / Q000473:pathology; D002539:Cerebral Arterial Diseases / Q000150:complications* / Q000378:metabolism / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications* / Q000378:metabolism / Q000473:pathology*; D007223:Infant; D007231:Infant, Newborn; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy
keywords: arteriopathy; incontinentia pigmenti; neonatal seizures; newborn; stroke