Cargando…
MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor: A Case Series
BACKGROUND AND OBJECTIVES: To characterize the clinical and neuroimaging phenotypes of patients with autoantibodies to γ-aminobutyric acid type A receptor (GABA(A)R). METHODS: Ten patients with autoantibodies against GABA(A)R from Huashan Hospital Autoimmune Encephalitis cohort were identified. We u...
Autores principales: | , , , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Lippincott Williams & Wilkins
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8958939/ https://www.ncbi.nlm.nih.gov/pubmed/35338092 http://dx.doi.org/10.1212/NXI.0000000000001158 |
_version_ | 1784677049637535744 |
---|---|
author | Deng, Bo Cai, Mengfei Qiu, Yue Liu, Xiaoni Yu, Hai Zhang, Xiang Huang, Huifen Zhao, Xiuhe Yang, Wenbo Dong, Siqi Jin, Lei Chu, Shuguang Chen, Xiangjun |
author_facet | Deng, Bo Cai, Mengfei Qiu, Yue Liu, Xiaoni Yu, Hai Zhang, Xiang Huang, Huifen Zhao, Xiuhe Yang, Wenbo Dong, Siqi Jin, Lei Chu, Shuguang Chen, Xiangjun |
author_sort | Deng, Bo |
collection | PubMed |
description | BACKGROUND AND OBJECTIVES: To characterize the clinical and neuroimaging phenotypes of patients with autoantibodies to γ-aminobutyric acid type A receptor (GABA(A)R). METHODS: Ten patients with autoantibodies against GABA(A)R 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 GABA(A)R expression were further investigated. RESULTS: The median age at onset of 10 patients (8 male patients and 2 female patients) with anti-GABA(A)R 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 GABA(A)R (Spearman ρ = 0.864, p = 0.001), the main target of autoantibodies. According to topology of lesions, patients with anti-GABA(A)R 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-GABA(A)R 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 GABA(A)R and reflected patients' disease severity and outcomes. |
format | Online Article Text |
id | pubmed-8958939 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-89589392022-03-29 MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor: A Case Series Deng, Bo Cai, Mengfei Qiu, Yue Liu, Xiaoni Yu, Hai Zhang, Xiang Huang, Huifen Zhao, Xiuhe Yang, Wenbo Dong, Siqi Jin, Lei Chu, Shuguang Chen, Xiangjun Neurol Neuroimmunol Neuroinflamm RESEARCH Article BACKGROUND AND OBJECTIVES: To characterize the clinical and neuroimaging phenotypes of patients with autoantibodies to γ-aminobutyric acid type A receptor (GABA(A)R). METHODS: Ten patients with autoantibodies against GABA(A)R 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 GABA(A)R expression were further investigated. RESULTS: The median age at onset of 10 patients (8 male patients and 2 female patients) with anti-GABA(A)R 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 GABA(A)R (Spearman ρ = 0.864, p = 0.001), the main target of autoantibodies. According to topology of lesions, patients with anti-GABA(A)R 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-GABA(A)R 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 GABA(A)R and reflected patients' disease severity and outcomes. Lippincott Williams & Wilkins 2022-03-25 /pmc/articles/PMC8958939/ /pubmed/35338092 http://dx.doi.org/10.1212/NXI.0000000000001158 Text en Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. |
spellingShingle | RESEARCH Article Deng, Bo Cai, Mengfei Qiu, Yue Liu, Xiaoni Yu, Hai Zhang, Xiang Huang, Huifen Zhao, Xiuhe Yang, Wenbo Dong, Siqi Jin, Lei Chu, Shuguang Chen, Xiangjun MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor: A Case Series |
title | MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor: A Case Series |
title_full | MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor: A Case Series |
title_fullStr | MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor: A Case Series |
title_full_unstemmed | MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor: A Case Series |
title_short | MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor: A Case Series |
title_sort | mri characteristics of autoimmune encephalitis with autoantibodies to gabaa receptor: a case series |
topic | RESEARCH Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8958939/ https://www.ncbi.nlm.nih.gov/pubmed/35338092 http://dx.doi.org/10.1212/NXI.0000000000001158 |
work_keys_str_mv | AT dengbo mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT caimengfei mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT qiuyue mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT liuxiaoni mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT yuhai mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT zhangxiang mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT huanghuifen mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT zhaoxiuhe mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT yangwenbo mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT dongsiqi mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT jinlei mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT chushuguang mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries AT chenxiangjun mricharacteristicsofautoimmuneencephalitiswithautoantibodiestogabaareceptoracaseseries |