Anti-Glutamate ε2 Receptor Antibody-Positive and Anti-N-Methyl-D-Aspartate Receptor Antibody-Negative Lobar Encephalitis Presenting as Global Aphasia and Swallowing Apraxia

Abstract

Background: Little is known about the difference between anti-N-methyl-D-aspartate receptor (NMDAR) antibody-positive encephalitis and anti-glutamate receptor (GluR) antibody-positive encephalitis. Objectives: To characterize anti-GluR antibody-positive encephalitis. Methods: We report a 33-year-old man with nonparaneoplastic anti-GluR ε2, ζ1 and δ2 antibody-positive and anti-NMDAR antibody-negative encephalitis, using neuropsychological tests and imaging studies including magnetic resonance imaging and single photon emission computed tomography (SPECT) with a 99mTc-ethylcysteinate dimer. Results: The patient exhibited global aphasia and swallowing apraxia (inability to transfer food to the pharyngeal cavity without sialorrhea). He was treated with 3 courses of corticosteroid pulse therapy and had recovered markedly 3 weeks after onset. Magnetic resonance diffusion-weighted images revealed hyperintensity in the bilateral frontal and left parietal cortices. Seven months later, a small area of hyperintensity in the left supramarginal gyrus remained. SPECT revealed hypoperfusion in extensive regions of the bilateral frontal lobes and left supramarginal gyrus. Thirteen months later, blood flow reduction was restricted to diffuse areas in the frontal lobes. Conclusions: Frontal lobar encephalitis without medial temporal involvement, marked cognitive impairment with a relatively preserved level of consciousness, and a favorable response to corticosteroid therapy, with nearly reversible cortical damage, may characterize anti-GluR antibody-positive encephalitis.

Keywords

• Anti-glutamate receptor antibodies
• Aphasia
• Lobar encephalitis
• N-methyl-D-aspartate receptor encephalitis
• Swallowing apraxia