RT Journal Article
SR Electronic
T1 MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e1158
DO 10.1212/NXI.0000000000001158
VO 9
IS 3
A1 Deng, Bo
A1 Cai, Mengfei
A1 Qiu, Yue
A1 Liu, Xiaoni
A1 Yu, Hai
A1 Zhang, Xiang
A1 Huang, Huifen
A1 Zhao, Xiuhe
A1 Yang, Wenbo
A1 Dong, Siqi
A1 Jin, Lei
A1 Chu, Shuguang
A1 Chen, Xiangjun
YR 2022
UL http://nn.neurology.org/content/9/3/e1158.abstract
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