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

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Abstract Background 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. Case presentation: A thirty-year-old female presented with a history of recurrent seizures and intellectual disability. Her delivery was through the vaginal route with unremarkable post-natal history. Her developmental milestones were normal until the age of three years when she was hospitalized for two weeks due to a febrile illness and generalized seizures, with full recovery. She earned a score of 14/60 on the Ravens Standard Progressive Matrices (RSPM) indicating an intellectual disability. The neurological examination revealed constructional apraxia, facial nerve upper motor neuron lesion (UMNL). A slow, sinuous writhing movement involving all fingers on the left hand was observed, and left spastic paresis. She underwent a computed tomography (CT) of the head which confirmed DDMS due to presence of right cerebral hemisphere volume loss with gross dilatation of the right lateral ventricle with ex-vacuo dilatation of the right lateral ventricle, right calvarial thickening and hyperpneumatization of the right frontal sinus. She was managed conservatively with anticonvulsants and had her seizure well controlled. Conclusion 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.

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