Dyke-Davidoff-Masson Syndrome: A Case Report and Review of Literature

Abstract

Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder that results from brain injury in intrauterine or early years of life. Prominent cortical sulci, dilated lateral ventricles, cerebral hemiatrophy, hyperpneumatization of the frontal sinus, and compensatory hypertrophy of the skull are the characteristic findings. We describe a male patient who presented with generalized tonic-clonic seizure and left-sided body weakness and neuroimaging findings of cerebral hemiatrophy, dilatation of right lateral ventricle, right frontal sinus hyperpneumatization, and asymmetric calvarial thickening. Knowledge of its features on imaging enables timely and accurate diagnosis, allowing appropriate management.

Keywords: cerebral atrophy; ddms; hemiplegia; seizures.

Figure 1. CT scan image showing the atrophy of the right cerebral hemisphere with the prominence of extra-axial cerebrospinal fluid (CSF) spaces (black arrow)

Figure 2. MRI (T1W) showing atrophic right cerebral hemisphere with dilatation of the right lateral ventricle and extra-axial cerebrospinal fluid (CSF) spaces (black arrows)

Figure 3. MRI showing hyperpneumatization of right frontal sinus (blue arrow) as compared to the left one