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

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 infec...

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Bibliographic Details
Published in:American Journal of Neuroradiology 2005-06, Vol.26 (6), p.1580-1582
Main Authors: Wolf, Nicole I, Kramer, Nikola, Harting, Inga, Seitz, Angelika, Ebinger, Friedrich, Poschl, Johannes, Rating, Dietz
Format: Article
Language:English
Subjects:
Biological and medical sciences
Cerebral Cortex - pathology
Electrocardiography. Vectocardiography
Electrodiagnosis. Electric activity recording
Encephalitis - etiology
Female
Humans
Incontinentia Pigmenti - complications
Incontinentia Pigmenti - diagnosis
Infant, Newborn
Investigative techniques, diagnostic techniques (general aspects)
Magnetic Resonance Imaging
Medical sciences
Miscellaneous
Necrosis
Nervous system
Neuropharmacology
Pediatrics
Pharmacology. Drug treatments
Radiodiagnosis. Nmr imagery. Nmr spectrometry
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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.
ISSN:0195-6108
1936-959X
1432-1920