Teaching Neuro Images : Clinical and neuroimaging features in Gorlin-Goltz syndrome

Abstract

An 18-year-old man presented with neuropsychomotor development delay since birth associated with multiple nevi and basal cell carcinomas (BCCs), which appeared in the third year of life (figure 1). Family history was unremarkable. Brain CT showed tentorium and falx cerebri calcification, associated with sphenoid hypoplasia (figure 2). Brain MRI disclosed a jaw odontogenic cyst (figure 2). Gorlin-Goltz syndrome (GGS) was diagnosed based on clinical and neuroimaging features.