Dyke Davidoff Masson Syndrome in a 34 Year Old Female: a Rare Case
DOI:
https://doi.org/10.24269/ijhs.v10i02.13310Abstract
Dyke–Davidoff–Masson Syndrome (DDMS) is a rare neurological disorder with fewer than 100 cases reported worldwide. It is characterized by cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of paranasal sinuses, often associated with hemiparesis, seizures, and developmental delay. Adult cases with symptoms persisting for more than three decades are exceptionally uncommon and offer valuable insight into long-term disease progression. Case Presentation: A 34-year-old woman was reported with a history of epileptic seizures since the age of 9 months, occurring 3–4 times per month and persisting into adulthood. The seizures were characterized by impaired consciousness accompanied by right-sided tonic–clonic motor manifestations and were preceded by a visual aura. The patient also exhibited right hemiparesis and delayed motor development. Neurological examination revealed right-sided central facial (cranial nerve VII) and hypoglossal (cranial nerve XII) paresis, right-sided limb spasticity, and visuospatial dysfunction. Non-contrast head computed tomography (CT) demonstrated left cerebral hemiatrophy, left lateral ventricular dilatation, left calvarial thickening, and hyperpneumatization of the left frontal sinus. This case was most appropriately classified as focal epilepsy of structural etiology secondary to DDMS, with a seizure type consistent with focal impaired consciousness seizure with observable manifestations.
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