| Summary: | Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological anomaly encompassing clinical features of seizures, contralateral hemiparesis, facial asymmetry, and intellectual dysfunction. Radiographic findings include cerebral hemiatrophy and ipsilateral calvarial thickening. We encountered an 11-year-old male who presented with new-onset seizures and a 4-year history of weakness in the abduction of his right arm, previously being managed as Erb's palsy. Brain MRI revealed atrophy of the left cerebral hemisphere with ipsilateral dilated ventricle and osseous thickening, consistent with the congenital form of DDMS. We present this case of an atypical presentation of DDMS.
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