A Case Series of Anti-Metabotropic Glutamate Receptor 2 Antibody-Related Diseases with Distinct Neurological Involvement

Zhanwei Zhang,1 Xiaolu Deng,1 Dandan Guo,1 Jie Zhao,2 Jian Li,2 Fang He,1,3 Lifen Yang,1,3 Jing Peng1,3 1Department of Pediatrics, Xiangya Hospital of Central South University, Changsha, Hunan Province, People’s Republic of China; 2Department of Neurosurgery, Xiangya Hospital of Central South Univer...

Full description

Saved in:
Bibliographic Details
Main Authors: Zhang Z, Deng X, Guo D, Zhao J, Li J, He F, Yang L, Peng J
Format: Article
Language:English
Published: Dove Medical Press 2025-04-01
Series:ImmunoTargets and Therapy
Subjects:
Online Access:https://www.dovepress.com/a-case-series-of-anti-metabotropic-glutamate-receptor-2-antibody-relat-peer-reviewed-fulltext-article-ITT
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849729599903105024
author Zhang Z
Deng X
Guo D
Zhao J
Li J
He F
Yang L
Peng J
author_facet Zhang Z
Deng X
Guo D
Zhao J
Li J
He F
Yang L
Peng J
author_sort Zhang Z
collection DOAJ
description Zhanwei Zhang,1 Xiaolu Deng,1 Dandan Guo,1 Jie Zhao,2 Jian Li,2 Fang He,1,3 Lifen Yang,1,3 Jing Peng1,3 1Department of Pediatrics, Xiangya Hospital of Central South University, Changsha, Hunan Province, People’s Republic of China; 2Department of Neurosurgery, Xiangya Hospital of Central South University, Changsha, Hunan Province, People’s Republic of China; 3Clinical Research Center for Children Neurodevelopmental Disabilities of Hunan Province, Changsha, People’s Republic of ChinaCorrespondence: Lifen Yang; Jing Peng, Department of Pediatrics, Xiangya Hospital of Central South University, 87 Xiangya Road, Changsha, Hunan Province, 410008, People’s Republic of China, Email yanglifen7@126.com; pengjing627@126.comBackground: Anti-metabotropic glutamate receptor 2 (mGluR2)-related diseases are rare autoimmune disorders of the central nervous system that primarily affect the cerebellum and are occasionally associated with malignancies.Methods: Data, including demographics, symptoms, blood and cerebrospinal fluid (CSF) tests, and brain magnetic resonance imaging (MRI), were retrospectively collected from two patients with informed consent at Xiangya Hospital from February 2024 to October 2024. Autoantibodies associated with autoimmune encephalitis were tested using cell-based assays. The literature describing anti-mGluR2 antibody-related diseases was searched for in PubMed and five cases were reviewed.Results: Two cases of anti-mGluR2 antibody-related diseases were reported: one with acute cerebellitis and the other with refractory seizures. Brain MRI showed cerebellar involvement in the cerebellitis patient. Anti-mGluR2 antibodies were detected in the serum but not in the CSF of both cases, and both responded well to immunotherapy. A review of five patients (all female, aged 3– 78 years) found four with cerebellar ataxia or cerebellitis and one with immune-related epilepsy. Common symptoms included dysarthria, gait instability, and gaze/nystagmus, while seizures were rare. MRI revealed cerebellar involvement in most cases. Anti-mGluR2 antibodies were present in the serum of all patients but only in the CSF of two. Three patients responded well to immunosuppressive treatment, and two had malignancies.Conclusion: Anti-mGluR2 antibody-related diseases are autoimmune disorders primarily characterized by ataxic manifestations, though seizures may also occur. The effectiveness of immunosuppressive treatment is uncertain and screening for tumors is necessary.Keywords: metabotropic glutamate receptor 2, antibody, cerebellitis, cerebellar ataxia, seizure
format Article
id doaj-art-5b64fe34cb6e47e0add8f1dca52fd213
institution DOAJ
issn 2253-1556
language English
publishDate 2025-04-01
publisher Dove Medical Press
record_format Article
series ImmunoTargets and Therapy
spelling doaj-art-5b64fe34cb6e47e0add8f1dca52fd2132025-08-20T03:09:11ZengDove Medical PressImmunoTargets and Therapy2253-15562025-04-01Volume 14465474101953A Case Series of Anti-Metabotropic Glutamate Receptor 2 Antibody-Related Diseases with Distinct Neurological InvolvementZhang ZDeng XGuo DZhao JLi JHe FYang LPeng JZhanwei Zhang,1 Xiaolu Deng,1 Dandan Guo,1 Jie Zhao,2 Jian Li,2 Fang He,1,3 Lifen Yang,1,3 Jing Peng1,3 1Department of Pediatrics, Xiangya Hospital of Central South University, Changsha, Hunan Province, People’s Republic of China; 2Department of Neurosurgery, Xiangya Hospital of Central South University, Changsha, Hunan Province, People’s Republic of China; 3Clinical Research Center for Children Neurodevelopmental Disabilities of Hunan Province, Changsha, People’s Republic of ChinaCorrespondence: Lifen Yang; Jing Peng, Department of Pediatrics, Xiangya Hospital of Central South University, 87 Xiangya Road, Changsha, Hunan Province, 410008, People’s Republic of China, Email yanglifen7@126.com; pengjing627@126.comBackground: Anti-metabotropic glutamate receptor 2 (mGluR2)-related diseases are rare autoimmune disorders of the central nervous system that primarily affect the cerebellum and are occasionally associated with malignancies.Methods: Data, including demographics, symptoms, blood and cerebrospinal fluid (CSF) tests, and brain magnetic resonance imaging (MRI), were retrospectively collected from two patients with informed consent at Xiangya Hospital from February 2024 to October 2024. Autoantibodies associated with autoimmune encephalitis were tested using cell-based assays. The literature describing anti-mGluR2 antibody-related diseases was searched for in PubMed and five cases were reviewed.Results: Two cases of anti-mGluR2 antibody-related diseases were reported: one with acute cerebellitis and the other with refractory seizures. Brain MRI showed cerebellar involvement in the cerebellitis patient. Anti-mGluR2 antibodies were detected in the serum but not in the CSF of both cases, and both responded well to immunotherapy. A review of five patients (all female, aged 3– 78 years) found four with cerebellar ataxia or cerebellitis and one with immune-related epilepsy. Common symptoms included dysarthria, gait instability, and gaze/nystagmus, while seizures were rare. MRI revealed cerebellar involvement in most cases. Anti-mGluR2 antibodies were present in the serum of all patients but only in the CSF of two. Three patients responded well to immunosuppressive treatment, and two had malignancies.Conclusion: Anti-mGluR2 antibody-related diseases are autoimmune disorders primarily characterized by ataxic manifestations, though seizures may also occur. The effectiveness of immunosuppressive treatment is uncertain and screening for tumors is necessary.Keywords: metabotropic glutamate receptor 2, antibody, cerebellitis, cerebellar ataxia, seizurehttps://www.dovepress.com/a-case-series-of-anti-metabotropic-glutamate-receptor-2-antibody-relat-peer-reviewed-fulltext-article-ITTmetabotropic glutamate receptor 2antibodycerebellitiscerebellar ataxiaseizure
spellingShingle Zhang Z
Deng X
Guo D
Zhao J
Li J
He F
Yang L
Peng J
A Case Series of Anti-Metabotropic Glutamate Receptor 2 Antibody-Related Diseases with Distinct Neurological Involvement
ImmunoTargets and Therapy
metabotropic glutamate receptor 2
antibody
cerebellitis
cerebellar ataxia
seizure
title A Case Series of Anti-Metabotropic Glutamate Receptor 2 Antibody-Related Diseases with Distinct Neurological Involvement
title_full A Case Series of Anti-Metabotropic Glutamate Receptor 2 Antibody-Related Diseases with Distinct Neurological Involvement
title_fullStr A Case Series of Anti-Metabotropic Glutamate Receptor 2 Antibody-Related Diseases with Distinct Neurological Involvement
title_full_unstemmed A Case Series of Anti-Metabotropic Glutamate Receptor 2 Antibody-Related Diseases with Distinct Neurological Involvement
title_short A Case Series of Anti-Metabotropic Glutamate Receptor 2 Antibody-Related Diseases with Distinct Neurological Involvement
title_sort case series of anti metabotropic glutamate receptor 2 antibody related diseases with distinct neurological involvement
topic metabotropic glutamate receptor 2
antibody
cerebellitis
cerebellar ataxia
seizure
url https://www.dovepress.com/a-case-series-of-anti-metabotropic-glutamate-receptor-2-antibody-relat-peer-reviewed-fulltext-article-ITT
work_keys_str_mv AT zhangz acaseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT dengx acaseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT guod acaseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT zhaoj acaseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT lij acaseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT hef acaseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT yangl acaseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT pengj acaseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT zhangz caseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT dengx caseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT guod caseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT zhaoj caseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT lij caseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT hef caseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT yangl caseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement
AT pengj caseseriesofantimetabotropicglutamatereceptor2antibodyrelateddiseaseswithdistinctneurologicalinvolvement