Dyke-Davidoff-Masson syndrome: an unusual cause of epilepsy

Abstract

Background: Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological condition with unknown global prevalence and incidence. The characteristic clinical features include facial asymmetry, seizures, and contralateral hemiplegia. Signature neuroimaging findings include unilateral brain volume loss, ventriculomegaly, compensatory bone hypertrophy resulting in cerebral hemiatrophy, calvarial thickening and hyperpneumatization of paranasal and frontal sinuses neuroimaging. Case Presentation: 4-year-old girl patient presented seizures (diagnosed as epilepsy) and complained of left-sided hemiplegia since 1 year ago. Neurological examination revealed left-sided spastic hemiplegia with brisk tendon reflexes and extensor planter response on the left side, increased reflexes physiology in the left limb, and positive Babinski on the left leg. MRI showed right cerebral hemiatrophy with ex vacuo dilatation of the right lateral, III and IV ventricles, with hyper pneumatization of the right ethmoid sinus and right left mastoid Aircell and elevation of the right petrous ridge, and falcine displacement to the right as far as +/- 7mm and blurring right hippocampus, suspected atrophy. Therapy, she got multiple anti-epileptic drugs. Conclusion: Diagnosis of DDMS is made by a triad of hemiplegia, contralateral hemiatrophy, seizures, and classical radiological features. Imaging is important in diagnosing this rare entity and differentiating it from other causes of cerebral hemiatrophy. The long-term prognosis is good, provided the clinical entity is recognized early and managed appropriately.