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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 26:1580-1582, June-July 2005
© 2005 American Society of Neuroradiology

Case Report
PEDIATRICS

Diffuse Cortical Necrosis in a Neonate with Incontinentia Pigmenti and an Encephalitis-Like Presentation

Nicole I. Wolf^a, Nikola Krämer, Inga Harting^c, Angelika Seitz^c, Friedrich Ebinger^a, Johannes Pöschl^b and Dietz Rating^a

^a Department of Pediatric Neurology, University Children’s Hospital Heidelberg, Germany
^b Department of Neonatology, University Children’s Hospital Heidelberg, Germany
^c Department of Neuroradiology, University Hospital Heidelberg, Heidelberg, Germany

Address correspondence to Nicole I. Wolf, MD, Department of Pediatric Neurology, University Children’s Hospital Heidelberg, Im Neuenheimer Feld 150, Heidelberg 69120, Germany

Summary: Incontinentia pigmenti is a rare neurocutaneous disorder that may present with neurologic symptoms, in addition to a characteristic vesicular rash within the first days of life. We describe a neonate girl presenting with a rash and an encephalopathy who was first thought to suffer from a viral infection and was only later recognized as being affected by incontinentia pigmenti. Cerebral MR imaging showed extensive cortical necrosis in the acute period. Incontinentia pigmenti should be included in the differential diagnosis of encephalopathy and cutaneous involvement in neonates, after a viral infection has been ruled out.

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				M. S. Cartwright, D. L. White, L. M. Miller III, and E. S. Roach Recurrent Stroke in a Child With Incontinentia Pigmenti J Child Neurol, May 1, 2009; 24(5): 603 - 605. [Abstract] [PDF]

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