Dyke-Davidoff-Masson Syndrome: A Case Report

Abstract

Background: Dyke-Davidoff-Masson syndrome (DDMS) is a neurological syndrome characterized by the presence of convulsions, facial asymmetry due to palsy of the facial nerve (CN VII), contralateral hemiplegia, and reduced intellectual capacity. The Case: We report a case of DDMS in a 20-year-old male who is a previously known case of generalized epilepsy on medication presenting with status epilepticus and initially managed by anticonvulsants. On admission, the seizures manifested again which required the patient to be sedated with injectable anesthetics and intubated. Clinical examination showed no focal neurological deficits or neurocutaneous markers. Imaging studies showed characteristic features of DDMS which were hemiatrophy of the right cerebrum with calvarial thickening, and sinuses showing hyperpneumatization on the same side as hemiatrophy. Previous history of such episodes had been recorded and the patient was kept on strict pharmacotherapy. Failure of adherence to these led to the current presentation. The diagnosis of DDMS was kept and the patient was treated conservatively with anticonvulsants and referred to a higher center for further management. Conclusion: DDMS, being a rare but important cause of refractory epilepsy, is easily missed on initial assessment and failure of adequate management leads to higher rates of morbidity and mortality associated with this syndrome. In cases with an atypical presentation, such as this one, a good background in radio-imaging and knowledge of the physical manifestations are required for final diagnosis.

Keywords

• Seizures
• Neuroimaging
• Anticonvulsants
• Cerebral atrophy
• Hemiplegia
• Dyke-Davidoff-Masson syndrome