Document Detail

Therapy resistant neonatal seizures, linear vesicular rash, and unusually early neuroradiological changes: incontinentia pigmenti: a case report, literature review and insight into pathogenesis.
MedLine Citation:
PMID:  17938957     Owner:  NLM     Status:  MEDLINE    
CASE PRESENTATION: A substance abusing G2P1 mother spontaneously delivered at term an appropriate for gestational age girl. Neonatal seizures appeared at 21 hours and empiric anticonvulsive and antimicrobial treatment was started. At 25 hours, first vesicles appeared. While routine evaluations remained normal, a head CT revealed multifocal ischemic injuries, and a later MRI showed multifocal petechiae and diffusion abnormalities in the corticospinal tracts. The clinical diagnosis of incontinentia pigmenti (stage 1) was secured by histopathology. Follow-up at 13 months showed global developmental delay. DISCUSSION: We discuss the unusually early bilateral, fronto-occipital corticomedullar ischemias (CT day 3). On the MR imaging (day 7) extensive symmetric cerebral corticomedullar destruction and diffusion sequences with corticospinal tracts abnormalities are seen, which then evolve (day 26) to extensive symmetric cerebral destruction. We review the literature, genetics, suspected pathophysiology and possible neonatal manifestation. CONCLUSION: Incontinentia pigmenti is rare and, therefore, diagnosis is frequently delayed. Nevertheless, in the setting of therapy refractory seizures, excluded infections, and linear vesicular rash, a high index of suspicion is needed. This is the first report of simultaneous corticomedullar involvement as early as the third day of life.
Gregor W Kaczala; Manuela A Messer; Ken J Poskitt; Juliette S Prendiville; Jane Gardiner; Christof Senger
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2007-10-16
Journal Detail:
Title:  European journal of pediatrics     Volume:  167     ISSN:  1432-1076     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  2008 Sep 
Date Detail:
Created Date:  2008-07-28     Completed Date:  2009-01-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  979-83     Citation Subset:  IM    
Department of Neonatology, Children's and Women's Health Center of British Columbia, Vancouver, BC, Canada.
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MeSH Terms
Anticonvulsants / therapeutic use
Exanthema / complications*
Incontinentia Pigmenti / complications*,  pathology*,  radiography
Infant, Newborn
Phenobarbital / therapeutic use
Seizures / complications*,  drug therapy
Reg. No./Substance:
0/Anticonvulsants; 50-06-6/Phenobarbital

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