Dyke-Davidoff-Masson Syndrome in Pediatrics: Case Report of Atypical Status Epilepticus and Therapeutic Challenges in the Caribbean.

Abstract

Dyke-Davidoff-Masson syndrome (DDMS), also known as cerebral hemiatrophy, is characterized by brain damage resulting in hypoplasia of one cerebral hemisphere. It is described as a rare disorder, primarily characterized by epileptic seizures and convulsions, as well as hemiparesis and cognitive impairments. We present the evolution of a child with DDMS and the neurodevelopmental milestones achieved from the onset of symptoms at 10 days of age to 3 years. Clinical features included persistent seizures and hemiparesis. Neuroimaging revealed right cerebral hemiatrophy with associated structural changes. The aim of this study is to present a case report of a pediatric patient with DDMS, detailing the clinical evaluation, therapeutic approaches, and disease progression, while describing the healthcare process and challenges associated with managing this rare condition in the Dominican Republic. Through this, we aim to improve the therapeutic strategies implemented in the region for the management of this infrequent condition and enhance the understanding of DDMS in the Caribbean.