Dyke-Davidoff-Masson Syndrome as a Rare Cause of Cerebral Hemiatrophy: Insights From a Case Series.

Dyke-Davidoff-Masson syndrome (DDMS) is an uncommon neurological condition marked by changes in the skeletal structure, cerebral hemiatrophy, and ventriculomegaly. Manifesting primarily in early life, DDMS presents with seizures, hemiplegia, facial asymmetry, and intellectual disabilities. There are congenital and acquired types of DDMS, with ischemia being the most common cause of the latter. Three cases are presented here to highlight the radiological and clinical characteristics of DDMS. The first case involves a 27-year-old male with generalized seizures and right-sided hemiparesis since childhood, along with developmental delays and facial asymmetry. The second case features a 20-year-old male with recurrent seizures and developmental delays. The third case involves a 25-year-old female with uncontrolled seizures and learning difficulties since childhood. The clinical and radiological characteristics of DDMS are demonstrated in all three cases, emphasizing the significance of early detection and differential diagnosis. Imaging techniques, such as computed tomography (CT) and magnetic resonance imaging (MRI), which demonstrate ipsilateral ventriculomegaly, brain atrophy, and associated bone abnormalities, are highly helpful in the diagnosis. Differential diagnoses include Sturge-Weber syndrome, linear nevus sebaceous syndrome (LNSS), Silver-Russell syndrome, Fishman syndrome, and Rasmussen encephalitis. Treatment aims at managing seizures and associated disabilities, with hemispherectomy considered for eligible cases. This case series underscores the significance of prompt diagnosis and multidisciplinary management in improving outcomes for individuals with DDMS.