Presentation
Progressive left side weakness
Patient Data
MRI demonstrates diffuse right cerebral hemiatrophy with ex-vacuo dilatation of the right lateral ventricle, right Sylvian fissure as well as cortical sulci. Reduction in volume of the ipsilateral cerebral peduncle due to Wallerian degeneration. Associated calvarial thickening with increased pneumatization of both frontal sinus and mastoid cells are also noted.
Associated crossed left cerebellar hemiatrophy with small cerebellum and prominent folia.
Case Discussion
Dyke–Davidoff–Masson syndrome (DDMS) is a rare disease characterized by cerebral atrophy secondary to an insult in utero or early childhood (acquired cases). Ipsilateral cerebellar atrophy occurs if the insult occurs before the age of 1 month while crossed cerebellar atrophy is seen if the insult occurs after the age of 1 month.