ISPUB.COM The Internet Journal of Pediatrics and Neonatology Volume 10 Number 2 1 of 4 Dyke Davidoff Masson syndrome in Children J Goyal, V Shah, S Rao, N Jindal Citation J Goyal, V Shah, S Rao, N Jindal. Dyke Davidoff Masson syndrome in Children. The Internet Journal of Pediatrics and Neonatology. 2008 Volume 10 Number 2. Abstract Cerebral hemiatrophy is not frequently encountered in clinical practice in children. We present here a case of 4 years old female child, who presented with seizures and weakness of left upper and lower limb and on MRI diagnosed to have Dyke Davidoff Masson Syndrome (DDMS). INTRODUCTION Dyke-Davidoff-Masson syndrome (DDMS) refers to atrophy or hypoplasia of one cerebral hemisphere (hemiatrophy) which is secondary to brain insult in fetal or early childhood period. It is not frequently encountered in clinical practice in children. We present here a case of a 4 year old female child, who presented with seizures and weakness of left upper and lower limb and on MRI was diagnosed to have DDMS. CASE REPORT A 4 years old female child was admitted to our hospital with complaints of weakness of left side of body for 2 years. Weakness was preceded by fever (indicating inflammatory etiology) and left sided recurrent tonic seizures which were relieved by anticonvulsant (carbamezapine). Since then the child had the same type of seizures even on regular medication. There was no history of significant perinatal and antenatal complications. Neurological examination revealed left sided spastic hemiparesis, brisk tendon reflexes and extensor planter on left side. There was no history of delayed mile stones. There were no neurocutaneous markers or asymmetry of face. Head circumference was 44 cm (<3rd percentile). Vision and hearing were normal and cranial nerves were intact. Hematological and CSF examination was normal. MRI of the patient showed severe atrophy of cerebral white matter on right side, thinning of cortical gyri and ex vacuo dilatation of right lateral ventricle. Atrophy of the right cerebral peduncle and thickening of right sided calvarium was also noted (Fig 1 & 2). Figure 1 Fig 1: FLAIR coronal section of MRI