Splenium Involvement in Oculodentodigital Dysplasia

Oculodentodigital Dysplasia (ODDD) is a rare autosomal dominant disorder causing abnormalities in the face, eyes, skeleton, and nervous system and is associated with mutations in GJA1. Magnetic resonance imaging (MRI) typically demonstrates hyperintensity on T2 weighted imaging in the parieto-occipital white matter and hypointensity of the subcortical grey matter structures. Computed tomography has described calcifications of the basal ganglia. We present a novel finding not described previously which is the involvement of the selenium of the corpus callosum.