A Case of Refractory Anti-GABAAR Encephalitis: Diagnostic Insights and Treatment Challenges
Abstract
A 59-year-old man developed olfactory dysfunction and behavioral changes that progressed to epilepsy. Brain magnetic resonance imaging (MRI) revealed multifocal cortico-subcortical T2-weighted fluid-attenuated inversion recovery (FLAIR) hyperintensities. Despite repeated plasmapheresis and immunotherapy with methylprednisolone, azathioprine, and cyclophosphamide, the response was limited. Follow-up MRI demonstrated recurrent cortical and subcortical lesions, sparing the brainstem, cerebellum, and basal ganglia. Gamma-aminobutyric acidA receptor (GABAAR) antibodies were subsequently detected in both serum and CSF, thus confirming the diagnosis. Anti-GABAAR encephalitis is an autoimmune disease characterized by refractory seizures and multifocal brain lesions. Multifocal lesions restricted to the cerebral hemispheres, sparing the brainstem, cerebellum, and basal ganglia, may suggest anti-GABAAR encephalitis.
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BibTeX
@article{mori2026case,
title = {{A Case of Refractory Anti-GABAAR Encephalitis: Diagnostic Insights and Treatment Challenges}},
author = {Takuma Mori and Satoshi Nozuma and Takahiro Hobara and Chikashi Yano and Yutaka Noguchi and Mika Dozono and Kento Kodama and Ryusei Nagata and Yujiro Higuchi and Makoto Hara and Hiroshi Takashima},
journal = {Internal Medicine},
year = {2026},
month = aug,
doi = {10.2169/internalmedicine.7435-26},
url = {https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7435-26/_article},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - A Case of Refractory Anti-GABAAR Encephalitis: Diagnostic Insights and Treatment Challenges
AU - Takuma Mori
AU - Satoshi Nozuma
AU - Takahiro Hobara
AU - Chikashi Yano
AU - Yutaka Noguchi
AU - Mika Dozono
AU - Kento Kodama
AU - Ryusei Nagata
AU - Yujiro Higuchi
AU - Makoto Hara
AU - Hiroshi Takashima
PY - 2026
DA - 2026/08/08
JO - Internal Medicine
DO - 10.2169/internalmedicine.7435-26
UR - https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7435-26/_article
AB - A 59-year-old man developed olfactory dysfunction and behavioral changes that progressed to epilepsy. Brain magnetic resonance imaging (MRI) revealed multifocal cortico-subcortical T2-weighted fluid-attenuated inversion recovery (FLAIR) hyperintensities. Despite repeated plasmapheresis and immunotherapy with methylprednisolone, azathioprine, and cyclophosphamide, the response was limited. Follow-up MRI demonstrated recurrent cortical and subcortical lesions, sparing the brainstem, cerebellum, and basal ganglia. Gamma-aminobutyric acidA receptor (GABAAR) antibodies were subsequently detected in both serum and CSF, thus confirming the diagnosis. Anti-GABAAR encephalitis is an autoimmune disease characterized by refractory seizures and multifocal brain lesions. Multifocal lesions restricted to the cerebral hemispheres, sparing the brainstem, cerebellum, and basal ganglia, may suggest anti-GABAAR encephalitis.
SN - 1349-7235
SN - 0918-2918
ER -