Dyke-Davidoff-Masson Syndrome as a rare congenital hemiatrophy: a case report Dyke-Davidoff-Masson Syndrome

Abstract

Introduction: Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition in childhood with very few cases reported in sub-Saharan Africa. Typically, the patient presents with facial asymmetry, seizures, and hemiparesis. Radiological findings include cerebral hemiatrophy, ipsilateral lateral ventricular dilatation, and hypertrophy of the calvarium and sinuses.

Case presentation: We present the report of a 3-year-old male with weakness of right upper and lower limbs, facial asymmetry, and seizures. Physical examination showed a well-nourished child with a squint of the right eye. There were brisk tendon reflexes with right-sided hemiplegia which is spastic with left limb preference. Computer tomography of the brain showed atrophy of the left cerebral hemisphere, ipsilateral lateral ventricular dilatation, and thickening of the ipsilateral cranium. The diagnosis of Dyke-Davidoff-Masson syndrome was made. He was treated with sodium valproate and lamotrigine and presently physiotherapy and he had remained seizure-free.

Conclusion: Dyke-Davidoff-Masson syndrome still affects children despite being a rare condition. There is a need for appropriate clinical and radiological assessment for the diagnosis of DDMS. Early identification and appropriate treatment will improve the general outcome of children with DDMS.