Radiographic features of the linear nevus sebaceous syndrome.

A rare neurocutaneous syndrome recognized recently is characterized by an epidermal nevus (linear nevus sebaceous of Jadassohn), seizures, and mental retardation [1-6]. In many cases the syndrome is associated with gross anatomic malformations of the central nervous system, eye, and tissues of mesenchymal origin, including the cardiovascular system and skeleton [2-10]. A great variety of radiographic manifestations have been reported, mostly in isolated case reports. However, none of the radiographic findings is characteristic for this syndrome; only correlation with clinical features, notably the skin lesion, can lead to the correct diagnosis. We recently examined two children with clinical featunes of the linear nevus sebaceous syndrome, one of whom had no evidence of any detectable anatomic cerebral, visceral, or skeletal anomaly and no abnormal radiographic findings. The second child had craniocerebral anomalies associated with significant radiographic manifestations of uncertain nature until the skin lesion was identified as a linear sebaceous nevus. (fig. 1). This case is briefly described below.