Abstract
We herein report a case of anti-gamma aminobutyric acid type A receptor antibody-associated encephalitis (anti-GABA(A)-RE) with progressive aphasia and generalized tonic-clonic seizures. Cerebral magnetic resonance imaging (MRI) showed cortical brain lesions coupled with hypermetabolism on fluorodeoxyglucose-positron emission tomography. After two courses of methylprednisolone pulse therapy, improvements in neurological symptoms without sequelae and the total disappearance of MRI lesions were observed. Upon encountering patients with refractory status epilepticus, multifocal cerebral MRI lesions, and suspected autoimmune encephalitis, especially in cases with thymoma, it would be prudent to suspect anti-GABA(A)-RE and consider the evaluation of anti-GABA(A) receptor antibody and methylprednisolone pulse therapy.