Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis

Anti-contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis is commonly characterized by limbic encephalitis with clinical symptoms of mental and behavior disorders, cognitive impairment, deterioration of memory, and epilepsy. The classical lesions reported are loca...

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Autores principales:	Pei Liu, Miao Bai, Chao Ma, Yaping Yan, Gejuan Zhang, Songdi Wu, Zunbo Li, Daidi Zhao, Kaixi Ren, Hongzeng Li, Jun Guo
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	autoimmune contactin-associated protein-like 2 encephalitis brainstem immunoglobulin G1 Immunologic diseases. Allergy RC581-607
Acceso en línea:	https://doaj.org/article/6e299da9679849bd8f09772e0db34253
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spelling	oai:doaj.org-article:6e299da9679849bd8f09772e0db342532021-11-11T05:30:54ZCase Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis1664-322410.3389/fimmu.2021.772763https://doaj.org/article/6e299da9679849bd8f09772e0db342532021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fimmu.2021.772763/fullhttps://doaj.org/toc/1664-3224Anti-contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis is commonly characterized by limbic encephalitis with clinical symptoms of mental and behavior disorders, cognitive impairment, deterioration of memory, and epilepsy. The classical lesions reported are located at the medial temporal lobe or hippocampus, whereas prominent brainstem lesions have not been addressed to date. Herein, we reported two patients mimicking progressive brainstem infarction with severe neurological manifestations. On brain magnetic resonance imaging (MRI), prominent brainstem lesions were noted, although multifocal lesions were also shown in the juxtacortical and subcortical white matters, basal ganglia, hippocampus, and cerebellar hemisphere. Unexpectedly and interestingly, both cases had detectable CASPR2 antibodies in sera, and an exclusive IgG1 subclass was documented in the further analysis. They were treated effectively with aggressive immunosuppressive therapies including corticosteroids, intravenous immunoglobulin G, and rituximab, with the first case achieving a rapid remission and the other undergoing a slow but gradual improvement. To the best of our knowledge, this is the first report on prominent brainstem involvement with definite MRI lesions in anti-CASPR2 antibody-associated autoimmune encephalitis, which helps to expand the clinical spectrum of this rare autoimmune disease and update the lesion patterns in the CNS.Pei LiuPei LiuMiao BaiChao MaYaping YanGejuan ZhangSongdi WuZunbo LiDaidi ZhaoKaixi RenHongzeng LiJun GuoFrontiers Media S.A.articleautoimmunecontactin-associated protein-like 2encephalitisbrainstemimmunoglobulin G1Immunologic diseases. AllergyRC581-607ENFrontiers in Immunology, Vol 12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	autoimmune contactin-associated protein-like 2 encephalitis brainstem immunoglobulin G1 Immunologic diseases. Allergy RC581-607
spellingShingle	autoimmune contactin-associated protein-like 2 encephalitis brainstem immunoglobulin G1 Immunologic diseases. Allergy RC581-607 Pei Liu Pei Liu Miao Bai Chao Ma Yaping Yan Gejuan Zhang Songdi Wu Zunbo Li Daidi Zhao Kaixi Ren Hongzeng Li Jun Guo Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis
description	Anti-contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis is commonly characterized by limbic encephalitis with clinical symptoms of mental and behavior disorders, cognitive impairment, deterioration of memory, and epilepsy. The classical lesions reported are located at the medial temporal lobe or hippocampus, whereas prominent brainstem lesions have not been addressed to date. Herein, we reported two patients mimicking progressive brainstem infarction with severe neurological manifestations. On brain magnetic resonance imaging (MRI), prominent brainstem lesions were noted, although multifocal lesions were also shown in the juxtacortical and subcortical white matters, basal ganglia, hippocampus, and cerebellar hemisphere. Unexpectedly and interestingly, both cases had detectable CASPR2 antibodies in sera, and an exclusive IgG1 subclass was documented in the further analysis. They were treated effectively with aggressive immunosuppressive therapies including corticosteroids, intravenous immunoglobulin G, and rituximab, with the first case achieving a rapid remission and the other undergoing a slow but gradual improvement. To the best of our knowledge, this is the first report on prominent brainstem involvement with definite MRI lesions in anti-CASPR2 antibody-associated autoimmune encephalitis, which helps to expand the clinical spectrum of this rare autoimmune disease and update the lesion patterns in the CNS.
format	article
author	Pei Liu Pei Liu Miao Bai Chao Ma Yaping Yan Gejuan Zhang Songdi Wu Zunbo Li Daidi Zhao Kaixi Ren Hongzeng Li Jun Guo
author_facet	Pei Liu Pei Liu Miao Bai Chao Ma Yaping Yan Gejuan Zhang Songdi Wu Zunbo Li Daidi Zhao Kaixi Ren Hongzeng Li Jun Guo
author_sort	Pei Liu
title	Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis
title_short	Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis
title_full	Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis
title_fullStr	Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis
title_full_unstemmed	Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis
title_sort	case report: prominent brainstem involvement in two patients with anti-caspr2 antibody-associated autoimmune encephalitis
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/6e299da9679849bd8f09772e0db34253
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Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis

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