Dyke-Davidoff-Masson Syndrome as a cause of hemiatrophy cerebral: About a clinical case

Abstract

Introduction: In the diagnostic approach to cases of cerebral hemiatrophy, Dyke-Davidoff-Masson syndrome should be considered, as it encompasses distinctive clinical and, especially, imaging features. In this regard, a clinical case will be presented, which started in childhood and was detected in adulthood.

Case presentation: A 46-year-old male with a relevant medical history of a herpes simplex infection at six months of age. Since then, he developed motor deficit of the left hemibody, intellectual disability, and refractory epilepsy. Neuroimaging evaluation revealed findings compatible with Dyke-Davidoff-Masson syndrome.

Discussion: Dyke-Davidoff-Masson syndrome (DDMS) was first described in 1933 as a condition of cerebral hemiatrophy. It is considered a rare epileptic syndrome that can present in a congenital and/or acquired form and is characterized by typical clinical and imaging findings. It may present with epileptic seizures, contralateral motor or sensory deficits, and intellectual disorders. On imaging, atrophy of one cerebral hemisphere, ipsilateral bone hypertrophy, and hyperpneumatization of the adjacent sinuses are commonly observed. Its treatment is symptomatic and based on a multidisciplinary approach.

Conclusions: A case of Dyke-Davidoff-Masson syndrome with onset in childhood is presented, which, in contrast to what is typically described, involved the right hemisphere.