Dyke-Davidoff-Masson Syndrome- A Case Report

Abstract

Dyke-Davidoff-Masson syndrome was first described in 1933 in series of 9 patients characterized by hemiparesis, seizures, facial asymmetry and mental retardation. It is referred to atrophy or hypoplasia of one cerebral hemisphere which is secondary to brain insult in fetal or early childhood period. Clinically these patients present with seizures, mental retardation, contralateral hemiparesis and facial asymmetry. The treatment is symptomatic and should target convulsions, hemiplegia, hemiparesis and learning difficulties. In case of intractable hemiplegia, hemispherectomy can be considered. Our case a 11 year female presented with complaints of recurrent generalized tonic clonic seizures since 3 years of age, mental retardation and weakness of right side of body. On examination, the child was having mental retardation and hence higher functions could not be assessed. On central nervous system examinations, there was hemiparesis on the right side. MRI brain showed left frontal, temporal, parietal and occipital lobe atrophy with mild prominence of temporal horn of left lateral ventricle and prominence of CSF spaces in vicinity. There was also hyperpneumatisation of left frontal sinus.