Refractory epilepsy accompanying acute encephalitis with multifocal cortical lesions: Possible autoimmune etiology

We report on a 14-year-old male suffering from acute encephalitis, whose clinical course met the criteria for acute encephalopathy with refractory, repetitive partial seizures (AERRPS). He presented with extremely refractory partial and secondary generalized seizures, and required high-dose barbiturate infusion therapy for 57 days under mechanical ventilation. Seven weeks after onset, the seizures were ameliorated by treatment with sodium bromide, carbamazepine, clobazam, and high-dose phenobarbital. Magnetic resonance imaging on day 14 of admission showed multifocal cortical legions scattered in the bilateral hemispheres; these disappeared on day 34. Diffuse and mild atrophy of the cerebral cortex, and moderate atrophy of the hippocampus, appeared by day 61. Serum anti-glutamate receptor ε2 autoantibodies were detected on day 2. The patient was discharged after 113 days of admission with intractable epilepsy, memory disability, and regression of intelligence. We discuss the etiological significance of the multifocal lesions, which are unusual findings on neuroimaging of AERRPS.