Dyke-Davidoff-Masson Syndrome: A Case Report in a Filipino Male Adolescent

Abstract

A previously well young Filipino boy presented with prolonged recurrent left focal to bilateral seizure. He was born of a non-consanguineous marriage, home-delivered vaginally, full term with no perinatal complications. He had normal growth and developmental milestones until at the age of 7 months when he developed febrile status epilepticus. Since then, he was left with residual left hemiplegia, dysarthria, cognitive delay and recurrent seizures occurring twice daily. He was poorly compliant on multiple anti-seizure medications. He was able to go to school and do activities of daily living with minimal supervision from his family. Symptoms of refractory seizures, hemiparesis, facial asymmetry, and intellectual disabilities along with brain imaging evidence of cerebral hemi-atrophy with compensatory calvarial thickening and subsequent hyperpneumatization is consistent with Dyke-Davidoff-Masson Syndrome (DDMS). A rare clinico-neuroradiologic condition occurring in fetal or early childhood period as a consequence of chronic brain insult. Diagnosis is established clinically with a characteristic cranial imaging finding. Hemi-spherectomy is the treatment of choice. This case will enlighten our mind as well as aid us in the prompt recognition of this uncommon syndrome with a classic clinico-radiologic presentation. Multidisciplinary intervention is essential, primarily to optimize seizure control as well as provide quality of life.