Opercular myoclonic-anarthric status epilepticus

Abstract

We report 3 cases of opercular myoclonic status epilepticus (OMASE), characterized by fluctuating cortical dysarthria without true aphasia associated with epileptic myoclonus involving bilaterally the glossopharyngeal musculature. In this syndrome, the inferior rolandic area of either one or the other hemisphere is involved by an epileptogenic lesion of various etiology. Ictally, clonic expression was consistent with epilepsia partialis continua (EPC) and bilaterally and symmetrically involved palatal muscles (cases 1-3), tongue (cases 2 and 3), lips and chin (case 3), and inferior jaw (case 1) due to bilateral projections of the inferior corticonuclear pathways. Postictally, the main clinical sign was pseudobulbar palsy, consistent with Todd's palsy. In our cases, OMASE was either of vascular (cases 1 and 2) or tumoral origin (case 3). In adulthood, early recognition of OMASE, although nonspecific, may be important for early management of carotid occlusive disease because it usually indicates an acute opercular infarction.