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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 23:490-492, March 2002
© 2002 American Society of Neuroradiology

PEDIATRICS

Sturge-Weber Disease with Repercussion on the Prenatal Development of the Cerebral Hemisphere

Pilar Portilla^a, Beatrice Husson^b, Pierre Lasjaunias^c and Pierre Landrieu^a

^a Department of Pediatric Neurology, CHU Paris Sud, Assistance Publique Hopitaux de Paris, Hopital de Bicêtre, France
^b Pediatric Radiology, CHU Paris Sud, Assistance Publique Hopitaux de Paris, Hopital de Bicêtre, France
^c Vascular Neuroradiology, CHU Paris Sud, Assistance Publique Hopitaux de Paris, Hopital de Bicêtre, France

Address reprint requests to Pierre Landrieu, Department of Pediatric Neurology, CHU Paris Sud, Assistance Publique Hopitaux de Paris, Hopital de Bicêtre 94270 France

Summary: Sturge-Weber syndrome was diagnosed in a neonate on basis of a characteristic port-wine stain. In the absence of any acute neurologic episode, MR images obtained when the infant was aged 3 months showed a typical pial vascular dysplasia, as well as prominent hypotrophy of the homolateral hemisphere. Areas suggesting the presence of developmental dysplasia of the cerebral mantel were found in association with the typical pial vascular anomaly. The prenatal effect of Sturge-Weber disease on normal brain development may best be explored by using a better evaluation with cerebral imaging shortly after birth.