Dyke Davidoff Masson Syndrome with Crossed Cerebellar Atrophy: A Case Report and Review of the Literature

Dyke Davidoff Masson Syndrome (DDMS) is characterized clinically by hemiplegia or hemiparesis, mental retardation, and epilepsy and radiologically by cerebral hemiatrophy, ipsilateral calvarial thickening, and dilation and increased aeration of the ipsilateral sinuses. Atrophy in the cerebral hemisphere may be accompanied by atrophy in the contralateral cerebellum. This condition is defined as crossed cerebellar atrophy (CCA). Although CCA’s pathogenesis is unclear, it has been associated with damage to the corticopontocerebellar pathways and neuronal connections between the cerebellum and contralateral cerebral hemisphere. Diffusion tensor imaging and conventional magnetic resonance imaging may help to demonstrate axonal connection pathways and make a diagnosis. Here we present a case of a 39-year-old female patient with clinical and radiological findings of DDMS associated with CCA.