Linear nevus sebaceous syndrome with hemimegalencephaly diagnosed in child in neonatal period
Jachowicz-Jeszka, J.; Juchniewicz, B.; Rotsztejn, H.; Wilkowski, J.ła.; Wendorff, J.
Polski Merkuriusz Lekarski Organ Polskiego Towarzystwa Lekarskiego 18(105): 310-313
2005
ISSN/ISBN: 1426-9686 PMID: 15997640 Document Number: 587558
The aim of this study was to present the case of the child with linear nevus sebaceous syndrome (LNSS) and hemimegalencephaly, diagnosed in neonatal period. It is one of the five epidermal nevus syndromes. Etiology of this multiorgan disease is still unknown. Most often LNSS consists of central nervous system, skeletal and ocular abnormalities. In present case different epileptic non-responsive seizures began in second week of life. In image exams hemimegalencephaly was found. EEG pattern showed generally discharges. The fits responded to vigabatrin. Psychomotor retardation was observed. Sometimes the skin lesion may be only manifestation of the syndrome. It is very important to perform early image and EEG examinations in LNSS.