Dyke-Davidoff-Masson Syndrome in a Tanzanian patient: a case report

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare neurological disorder resulting from an insult to the developing brain in utero or during early life which is characterized by refractory seizures and a wide variety of deficits. Due to its rarity, the condition is often overlooked although a detailed history and imaging can help distinguish it from other similar conditions and provide early diagnosis. A 30-year-old African female presented with a history of recurrent seizures and intellectual disability. She was born via vaginal delivery, and her post-natal history was unremarkable. Her developmental milestones were normal until the age of three, when she was hospitalized for two weeks due to a febrile illness accompanied by generalized seizures, from which she fully recovered. On cognitive assessment, she scored 14 out of 60 on the Ravens Standard Progressive Matrices (RSPM), indicating an intellectual disability. Neurological examination revealed constructional apraxia, an upper motor neuron lesion (UMNL) affecting the facial nerve, and left-sided spastic paresis. Additionally, a slow, sinuous writhing movement involving all the fingers of her left hand was observed. A computed tomography (CT) scan of her head confirmed a diagnosis of Dyke-Davidoff-Masson syndrome (DDMS). The scan showed significant volume loss in the right cerebral hemisphere, gross dilation of the right lateral ventricle with ex-vacuo dilatation, right calvarial thickening, and hyperpneumatization of the right frontal sinus. She was managed conservatively with anticonvulsants, which effectively controlled her seizures. In the setting of recurrent seizures, intellectual disability, hemiparesis, or facial asymmetry, a clinician should have a high index of suspicion for DDMS. Late diagnosis is often related to intractable seizures to anticonvulsants, necessitating hemispherectomy, which is not a readily available option in limited resource settings.