P-EP008. Dyke-Davidoff-Masson syndrome: A case report in a Filipino male adolescent

Abstract

Introduction. A 17-year-old male who presented with recurrent left focal seizure with secondary generalization. He was born of a nonconsanguineous marriage, home-delivered vaginally, full term with no perinatal complications. He had normal growth and developmental milestones until at 7-month-old when he had febrile status epilepticus. Since then he was left with residual left hemiplegia, dysarthria, cognitive delay and recurrent seizure occurring twice daily. He was able to go to school with minimal supervision from his family for hygiene and safety. Refractory seizures, hemiparesis, facial asymmetry, and intellectual disabilities along with brain imaging evidence of cerebral hemiatrophy 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 characteristic brain imaging findings. Multidisciplinary intervention is essential, primarily to optimize seizure control as well as provide quality of life.