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May 2022; 9 (3) Research ArticleOpen Access

MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor

A Case Series

Bo Deng, View ORCID ProfileMengfei Cai, Yue Qiu, Xiaoni Liu, Hai Yu, Xiang Zhang, Huifen Huang, Xiuhe Zhao, Wenbo Yang, Siqi Dong, Lei Jin, View ORCID ProfileShuguang Chu, View ORCID ProfileXiangjun Chen
First published March 25, 2022, DOI: https://doi.org/10.1212/NXI.0000000000001158
Bo Deng
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: dengbo1991@outlook.com
Mengfei Cai
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • ORCID record for Mengfei Cai
  • For correspondence: cmfdream@foxmail.com
Yue Qiu
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: 12301010084@fudan.edu.cn
Xiaoni Liu
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: xiaoniliu13@fudan.edu.cn
Hai Yu
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: amplatzer@hotmail.com
Xiang Zhang
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: zhangxiang1213@hotmail.com
Huifen Huang
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: wuyumomo@aliyun.com
Xiuhe Zhao
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: zhaoxiuhe@126.com
Wenbo Yang
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: 644729499@qq.com
Siqi Dong
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: 540694953@qq.com
Lei Jin
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: jin0913lei@163.com
Shuguang Chu
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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  • For correspondence: chushu1018@hotmail.com
Xiangjun Chen
From the Department of Neurology (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Huashan Hospital, Fudan University, Shanghai; National Center for Neurological Disorders (B.D., Y.Q., X.L., H.Y., X. Zhang, W.Y., S.D., L.J., X.C.), Shanghai, China; Department of Neurology (M.C.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands; Institute of Neurology (X.L., X. Zhang, X.C.), Fudan University, Shanghai; Department of Neurology (H.H.), Lishui Hospital, Zhejiang University School of Medicine; Department of Neurology (X. Zhao), Qilu Hospital, Shandong University, Jinan; Department of Radiology (S.C.), Shanghai East Hospital, Tongji University School of Medicine; and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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Full PDF
Citation
MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor
A Case Series
Bo Deng, Mengfei Cai, Yue Qiu, Xiaoni Liu, Hai Yu, Xiang Zhang, Huifen Huang, Xiuhe Zhao, Wenbo Yang, Siqi Dong, Lei Jin, Shuguang Chu, Xiangjun Chen
Neurol Neuroimmunol Neuroinflamm May 2022, 9 (3) e1158; DOI: 10.1212/NXI.0000000000001158

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This article has a correction. Please see:

  • Errata - November 01, 2022
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    Figure 1 Characteristics of Brain MRI in Anti-GABAAR Encephalitis

    Note that multiple cortical and subcortical lesions show hyperintense signal on FLAIR imaging (A.a), hypointense signal on T1-weighted imaging without evident mass effect and gadolinium enhancement (A.b, A.c), and no significant restricted diffusion on diffusion-weighted imaging and apparent diffusion coefficient maps (A.d, A.e). Long-term follow-up FLAIR scans in 1 representative patient (patient 1) show confluent lesions predominantly involved bilateral frontal and temporal lobes at the acute stage (B.a, B.b). Some lesions disappeared, but new multifocal lesions emerged 1.5 months later (C.a, C.b). Seven months after onset, most intracranial lesions at acute stage resolved, but signs of cortical atrophy and enlarged lateral ventricles appeared (D.a, D.b). Three years after onset, this patient had stable clinical symptoms without any lesion but dilated lateral ventricles (red circle) and brain atrophy predominantly involved in temporal and frontal lobes (red arrow) (E.a, E.b). Of note, the FLAIR scans at the follow-ups were registered in the native FLAIR space at baseline to ensure that the scans from 4 time points were comparable anatomically with a side-by-side inspection. FLAIR = fluid-attenuated inversion recovery; GABAAR = γ-aminobutyric acid type A receptor.

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    Figure 2 Two Types of Representative Lesion Distribution Patterns in Anti-GABAAR Encephalitis

    Type 1: confluent type (A); type 2: spotted type (B). Note that patients with both types had bilateral but asymmetric lesion involvement in cingulate gyri (red arrow). GABAAR = γ-aminobutyric acid type A receptor.

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    Figure 3 Lesion Probability Map of Anti-GABAAR Encephalitis

    Note that limbic (especially bilateral anterior cingulate gyri, white arrow), frontal, and temporal lobes were dominantly involved. GABAAR = γ-aminobutyric acid type A receptor.

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    Figure 4 Lateral and Medial Views of GABRA1, GABRB3, andGABRR2 Gene Expression Data Projected Onto the FreeSurfer Cortical Regions

    Expression level was log2 transformed. Note the high expression of GABAB3 in limbic, frontal, and temporal lobes but a relative low expression in parietal and occipital lobes.

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