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Pre- and postnatal imaging of early cerebral damage in Sturge-Weber syndrome

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Abstract

A case of prenatal diagnosis of Sturge-Weber syndrome associated with polymicrogyria is reported. The diagnosis was based on a unique association with unilateral hemispheric gyriform calcification, focal hemispheric atrophy and white matter changes on prenatal imaging including ultrasound and MRI. Polymicrogyria, which is exceptionally associated with Sturge-Weber syndrome, is suggestive of and reinforces the hypothesis of early impairment of the cerebral microvasculature related to leptomeningeal angioma, which may lead to abnormal cerebral development as early as the second trimester of pregnancy.

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References

  1. Portilla P, Husson B, Lasjaunias P et al (2002) Sturge-Weber disease with repercussion on the prenatal development of the cerebral hemisphere. AJNR Am J Neuroradiol 23:490–492

    PubMed  Google Scholar 

  2. Avez-Couturier J, Auvin S, Cuisset JM et al (2006) Status epilepticus in a 2-month-old infant. Arch Pediatr 13:1525

    Article  Google Scholar 

  3. Comi AM (2006) Advances in Sturge-Weber syndrome. Curr Opin Neurol 19:124–128

    Article  PubMed  Google Scholar 

  4. Terdjman P, Aicardi J, Sainte-Rose C et al (1991) Neuroradiological findings in Sturge-Weber syndrome and isolated pial angiomatosis. Neuropediatrics 22:115–120

    Article  PubMed  CAS  Google Scholar 

  5. Adamsbaum C, Pinton F, Rolland Y et al (1996) Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations. Pediatr Radiol 26:759–762

    Article  PubMed  CAS  Google Scholar 

  6. Hertz Pannier L, Laffond C, Dulac O et al (2009) Diagnostic précoce par IRM de l’angiome pial dans la maladie de Sturge Weber. J Radiol 90:1592. http://pe.sfrnet.org/ModuleConsultationPoster/posterDetail.aspx?intIdPoster=3929

    Google Scholar 

  7. Murakami N, Morioka T, Suzuki SO et al (2012) Focal cortical dysplasia type IIa underlying epileptogenesis in patients with epilepsy associated with Sturge-Weber syndrome. Epilepsia 53:e184–188

    Article  PubMed  Google Scholar 

  8. Stranzinger E, Huisman M (2007) Sturge-Weber syndrome. Early manifestation and visualization of disease course. Radiologe 47:1126–1130

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Pediatric and Fetal Imaging, Hôpital Femme Mère Enfant, 59, boulevard Pinel, 69677, Lyon-Bron, France

    Maud Cagneaux, Vincent Paoli & Laurent Guibaud

  2. Pediatric Neurology Department, Hôpital Femme Mère Enfant, 59, boulevard Pinel, 69677, Lyon-Bron, France

    Gaëlle Blanchard & Dorothée Ville

Authors
  1. Maud Cagneaux
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  2. Vincent Paoli
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  3. Gaëlle Blanchard
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  4. Dorothée Ville
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  5. Laurent Guibaud
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Correspondence to Laurent Guibaud.

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Cagneaux, M., Paoli, V., Blanchard, G. et al. Pre- and postnatal imaging of early cerebral damage in Sturge-Weber syndrome. Pediatr Radiol 43, 1536–1539 (2013). https://doi.org/10.1007/s00247-013-2743-9

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  • DOI: https://doi.org/10.1007/s00247-013-2743-9

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