“Erratic” complex partial status epilepticus as a presenting feature of MELAS

Patients with the syndrome of mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) may rarely present with complex partial status epilepticus (CPSE) at clinical onset. We report on a 38-year-old woman with MELAS presenting with multifocal CPSE and periodic lateralized epileptiform discharges (PLEDs) on EEG during her first strokelike episode. CT scan documented a right temporo-parieto-occipital strokelike lesion. EEG showed prolonged seizure discharges with alternating focus over the temporo-occipital and frontotemporal regions of the right hemisphere; moreover, right frontotemporal PLEDs were evident when the seizure activity was localized in the temporo-occipital region. The electroclinical status and CT findings normalized gradually on carbamazepine therapy. The four other patients with MELAS described in the literature as presenting with CPSE showed unifocal epileptic discharges on EEG. We report for the first time a case in which multifocal CPSE is the presenting feature of MELAS. Our findings document the multifocality of neuronal hyperexcitability in the context of the cerebral strokelike lesion in this syndrome.