Presentation of Dyke-Davidoff-Masson Syndrome in a 32-year-old female: Report of a rare case with a literature review

Document Type

Case Report


Paediatrics and Child Health


Dyke-Davidoff-Masson syndrome (DDMS) is a rare congenital or acquired neurological disorder that most commonly affects the pediatric population but is also rarely reported in adults. DDMS results from brain injury in the intrauterine or early years of life. It is characterized by prominent cortical sulci, hyperpneumatization of the frontal sinus, unilateral cerebral hemiatrophy with ventricular dilation, and associated bony thickness of the cranial vault. Seizures and asymmetric hemiparesis are the most consistent findings in DDMS with facial asymmetry and mental retardation widely reported. Herein, we report a case of a 32-year-old female patient with DDMS presenting with a history of seizure and right-sided hemiparesis. Neuroimaging findings showed asymmetric cerebral encephalomalacia and gliosis with ex vacuo ventricular dilatation and calvarial diploic space widening. Our case report is unique in the sense that our patient presented with DDMS in adulthood with no signs of mental retardation or history of seizures during childhood and well-controlled seizures on monotherapy. Given the adult presentation of DDMS is unusual and rarely reported in the medical literature, our case report will help physicians to keep DDMS high on differential diagnoses in such cases. Awareness of the clinical features of DDMS on imaging can facilitate a timely and accurate diagnosis, thereby enabling appropriate and prompt management.


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