Case Reports

European Journal of Pediatrics

, Volume 144, Issue 3, pp 290-294

Neuroradiological findings in Jadassohn nevus phakomatosis: a report of four cases

  • J. S. H. VlesAffiliated withDepartment of Paediatrics, Katholieke Universiteit Leuven
  • , P. DegraeuweAffiliated withDepartment of Paediatrics, Katholieke Universiteit Leuven
  • , P. De CockAffiliated withDepartment of Paediatrics, Katholieke Universiteit Leuven
  • , P. CasaerAffiliated withDepartment of Paediatrics, Katholieke Universiteit Leuven

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Abstract

Four patients with Jadassohn nevus phakomatosis (linear nevus sebaceus syndrome, Schimmelpenning-Feuerstein syndrome) are described. Neuroradiological findings consisted of skull asymmetry shown by X-rays and hemimegalencephaly with ventricular system asymmetry on CT. Three of the patients showed, in addition, a widened insula. In two patients areas of increased density suggesting calcium deposits were seen. Skull asymmetry was progressive during infancy in two of the patients. It is presumed that these findings are secondary to the progression of the underlying central nervous system disorder.

Key words

Jadassohn nevus phakomatosis Linear nevus sebaceous syndrome Schimmelpenning-Feuerstein syndrome Skull X-rays Hemimegalencephaly