Successful combination immunotherapy of anti-gamma aminobutyric acid (GABA)A receptor antibody-positive encephalitis with extensive multifocal brain lesions

Abstract

A 78-year old woman who presented with akinetic mutism was admitted to our hospital. Brain MRI showed multifocal increased T2/FLAIR signal with extensive cortical-subcortical involvement. We suspected autoimmune encephalitis and the patient received methylprednisolone pulse. Her conscious level gradually recovered, but later relapsed again and presented with refractory status epilepticus. We treated her with intravenous immunoglobulin, plasma exchange and pulsed cyclophosphamide, with satisfactory response. A brain biopsy showed perivascular lymphocytic infiltrates and reactive gliosis. Anti-gamma aminobutyric acid (GABA)A receptor antibodies test came back to be positive after her recovery, and the diagnosis of anti-GABAA receptor antibody-positive encephalitis was made. This is a very rare case where brain biopsies were performed in a patient with anti-GABAA receptor antibody-positive encephalitis. (Rinsho Shinkeigaku (Clin Neurol) 2017;57:436-440).