PT - JOURNAL ARTICLE AU - Deng, Bo AU - Cai, Mengfei AU - Qiu, Yue AU - Liu, Xiaoni AU - Yu, Hai AU - Zhang, Xiang AU - Huang, Huifen AU - Zhao, Xiuhe AU - Yang, Wenbo AU - Dong, Siqi AU - Jin, Lei AU - Chu, Shuguang AU - Chen, Xiangjun TI - MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor AID - 10.1212/NXI.0000000000001158 DP - 2022 May 01 TA - Neurology - Neuroimmunology Neuroinflammation PG - e1158 VI - 9 IP - 3 4099 - http://nn.neurology.org/content/9/3/e1158.short 4100 - http://nn.neurology.org/content/9/3/e1158.full SO - Neurol Neuroimmunol Neuroinflamm2022 May 01; 9 AB - Background and Objectives To characterize the clinical and neuroimaging phenotypes of patients with autoantibodies to γ-aminobutyric acid type A receptor (GABAAR).Methods Ten patients with autoantibodies against GABAAR from Huashan Hospital Autoimmune Encephalitis cohort were identified. We used MRI assessments and clinical examinations to summarize major clinical profile and visualize and quantify lesion distribution features. The relationship between clinical features, neuroimaging phenotypes, and topology of GABAAR expression were further investigated.Results The median age at onset of 10 patients (8 male patients and 2 female patients) with anti-GABAAR encephalitis was 41.5 years (range: 17–73 years). All patients had prominent seizures and multifocal spotted or confluent lesions involved in limbic, frontal, and temporal lobes on brain MRI. Bilateral but asymmetric lesions in cingulate gyri were observed in all patients. These involved lesions could change dynamically with immunotherapies and relapse. Distribution of patients' brain MRI lesions was positively correlated with gene expression level of β3 subunit–containing GABAAR (Spearman ρ = 0.864, p = 0.001), the main target of autoantibodies. According to topology of lesions, patients with anti-GABAAR encephalitis could be classified into 2 clinical-radiological types: confluent type with bilateral confluent lesions involved in almost all limbic, frontal, and temporal lobes and spotted type with multiple scattered small-to-medium patchy lesions. Patients with confluent type exhibited worse clinical presentations and outcomes when compared with those with spotted type (maximum modified Rankin scale [mRS]: 5 [5–5] vs 3.5 [3–4], respectively, p = 0.008; follow-up mRS: 4 [2–6] vs 0.5 [0–1], respectively, p = 0.016).Discussion Anti-GABAAR encephalitis has distinctive neuroimaging phenotype. Cingulate gyri were frequently involved in this disorder. The topology of lesions might be associated with the distribution of β3 subunit–containing GABAAR and reflected patients' disease severity and outcomes.AE=autoimmune encephalitis; AED=antiepileptic drug; CBA=cell-based assay; Caspr2=contactin-associated protein-like 2; FLAIR=fluid-attenuated inversion recovery; GABAAR=γ-aminobutyric acid type A receptor; GABABR=γ-aminobutyric acid type B receptor; GAD=glutamic acid decarboxylase; HHV6=human herpesvirus 6; ICU=intensive care unit; LGI1=leucine-rich glioma-inactivated 1; MNI=Montreal Neurological Institute; MOGAD=myelin oligodendrocyte glycoprotein-antibody-associated disease; mRS=modified Rankin scale; RT=room temperature