Paraneoplastic cerebellar ataxia and antibodies to metabotropic glutamate receptor 2
Objective To report the presence of a new neuronal surface antibody against the metabotropic glutamate receptor 2 antibody (mGluR2-Ab) in 2 patients with paraneoplastic cerebellar ataxia. Methods mGluR2-Abs were initially characterized by immunohistochemistry on the rat brain and confirmed by immuno...
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| Veröffentlicht in: | Neurology : neuroimmunology & neuroinflammation 2020-03, Vol.7 (2), p.e658-e658 |
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| creator | Ruiz-García, Raquel Martínez-Hernández, Eugenia Joubert, Bastien Petit-Pedrol, Mar Pajarón-Boix, Elena Fernández, Verónica Salais, Lidia del Pozo, María Armangué, Thais Sabater, Lidia Dalmau, Josep Graus, Francesc |
| description | Objective To report the presence of a new neuronal surface antibody against the metabotropic glutamate receptor 2 antibody (mGluR2-Ab) in 2 patients with paraneoplastic cerebellar ataxia. Methods mGluR2-Abs were initially characterized by immunohistochemistry on the rat brain and confirmed by immunofluorescence on HEK293 cells transfected with mGluR2. Additional studies included analysis of potential cross-reactivity with other mGluRs, expression of mGluR2 in patients' tumors, and the effects of mGluR2-Abs on cultures of rat hippocampal neurons. Results Patient 1 was a 78-year-old woman with progressive cerebellar ataxia with an initial relapsing-remitting course who developed a small-cell tumor of unknown origin. Patient 2 was a 3-year-old girl who presented a steroid-responsive acute cerebellitis preceding the diagnosis of an alveolar rhabdomyosarcoma. Patients' serum and CSF showed a characteristic immunostaining of the hippocampus and cerebellum in rat brain sections and immunolabeled the cell surface of live rat hippocampal neurons. HEK293 cells transfected with mGluR1, 2, 3, and 5 confirmed that patients' antibodies only recognized mGluR2. mGluR2-Abs were not detected in 160 controls, 120 with paraneoplastic, autoimmune, or degenerative ataxias, and 40 with autoimmune encephalitis and antibodies against mGluR5 or unknown antigens. Expression of mGluR2 in tumors was confirmed by immunohistochemistry using a commercial mGluR2-Ab. Incubation of live rat hippocampal neurons with CSF of patient 2 did not modify the density of surface mGluR2 clusters. Conclusions mGluR2-Abs are a novel biomarker of paraneoplastic cerebellar ataxia. The potential pathogenic effect of the antibodies is not mediated by downregulation or internalization of neuronal surface mGluR2. |
| doi_str_mv | 10.1212/NXI.0000000000000658 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmed_primary_31826987</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2325301746</sourcerecordid><originalsourceid>FETCH-LOGICAL-c5190-d617af19f22ce1d600bbb3aa7a03e42897f60f34e012dc9ab73b780c0afd1f5c3</originalsourceid><addsrcrecordid>eNqNkl-L1DAUxYMo7rLuNxDpoyBd86dp2hdBhnVdWNSHFXwLN-ntTrRtapI6-u03ZdZx9MlASCC_c3JvTgh5zugF44y__vDl-oIej1o2j8gpF4KXqmH88dH-hJzH-DUzjEupavWUnAjW8Lpt1Cm5_QQBJvTzADE5W1gMaHAYIBSQ4KeDAqYuz-SM7xzGIvlixATGp-DnLLgblgQjJCwCWpyTDwV_Rp70MEQ8f1jPyOd3l7eb9-XNx6vrzdub0krW0rKrmYKetT3nFllXU2qMEQAKqMCKN63qa9qLCnPlnW3BKGFUQy2FvmO9tOKMvNn7zosZsbM4pQCDnoMbIfzSHpz--2RyW33nf-i6rYSoZTZ4-WAQ_PcFY9Kji3ZtPz_JEjUXXArKVFVntNqjNvgYA_aHaxjVayY6Z6L_zSTLXhyXeBD9TiADr_bADo3vo3U4WTxg2UXytpVMrIarXfP_9MYlSM5PG79M6U8DOz8kDPHbsOww6C3CkLY6N9moirKSU07pqi_XH0PFPYoxuYg</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2325301746</pqid></control><display><type>article</type><title>Paraneoplastic cerebellar ataxia and antibodies to metabotropic glutamate receptor 2</title><source>MEDLINE</source><source>DOAJ Directory of Open Access Journals</source><source>Journals@Ovid Complete</source><source>Wolters Kluwer Open Health</source><source>Web of Science - Science Citation Index Expanded - 2020<img src="https://exlibris-pub.s3.amazonaws.com/fromwos-v2.jpg" /></source><source>EZB-FREE-00999 freely available EZB journals</source><source>PubMed Central</source><creator>Ruiz-García, Raquel ; Martínez-Hernández, Eugenia ; Joubert, Bastien ; Petit-Pedrol, Mar ; Pajarón-Boix, Elena ; Fernández, Verónica ; Salais, Lidia ; del Pozo, María ; Armangué, Thais ; Sabater, Lidia ; Dalmau, Josep ; Graus, Francesc</creator><creatorcontrib>Ruiz-García, Raquel ; Martínez-Hernández, Eugenia ; Joubert, Bastien ; Petit-Pedrol, Mar ; Pajarón-Boix, Elena ; Fernández, Verónica ; Salais, Lidia ; del Pozo, María ; Armangué, Thais ; Sabater, Lidia ; Dalmau, Josep ; Graus, Francesc</creatorcontrib><description>Objective To report the presence of a new neuronal surface antibody against the metabotropic glutamate receptor 2 antibody (mGluR2-Ab) in 2 patients with paraneoplastic cerebellar ataxia. Methods mGluR2-Abs were initially characterized by immunohistochemistry on the rat brain and confirmed by immunofluorescence on HEK293 cells transfected with mGluR2. Additional studies included analysis of potential cross-reactivity with other mGluRs, expression of mGluR2 in patients' tumors, and the effects of mGluR2-Abs on cultures of rat hippocampal neurons. Results Patient 1 was a 78-year-old woman with progressive cerebellar ataxia with an initial relapsing-remitting course who developed a small-cell tumor of unknown origin. Patient 2 was a 3-year-old girl who presented a steroid-responsive acute cerebellitis preceding the diagnosis of an alveolar rhabdomyosarcoma. Patients' serum and CSF showed a characteristic immunostaining of the hippocampus and cerebellum in rat brain sections and immunolabeled the cell surface of live rat hippocampal neurons. HEK293 cells transfected with mGluR1, 2, 3, and 5 confirmed that patients' antibodies only recognized mGluR2. mGluR2-Abs were not detected in 160 controls, 120 with paraneoplastic, autoimmune, or degenerative ataxias, and 40 with autoimmune encephalitis and antibodies against mGluR5 or unknown antigens. Expression of mGluR2 in tumors was confirmed by immunohistochemistry using a commercial mGluR2-Ab. Incubation of live rat hippocampal neurons with CSF of patient 2 did not modify the density of surface mGluR2 clusters. Conclusions mGluR2-Abs are a novel biomarker of paraneoplastic cerebellar ataxia. The potential pathogenic effect of the antibodies is not mediated by downregulation or internalization of neuronal surface mGluR2.</description><identifier>ISSN: 2332-7812</identifier><identifier>EISSN: 2332-7812</identifier><identifier>DOI: 10.1212/NXI.0000000000000658</identifier><identifier>PMID: 31826987</identifier><language>eng</language><publisher>PHILADELPHIA: American Academy of Neurology</publisher><subject>Aged ; Animals ; Autoantibodies - immunology ; Autoantibodies - metabolism ; Biomarkers, Tumor - immunology ; Biomarkers, Tumor - metabolism ; Carcinoma, Neuroendocrine - complications ; Carcinoma, Neuroendocrine - immunology ; Carcinoma, Neuroendocrine - metabolism ; Cerebellar Ataxia - etiology ; Cerebellar Ataxia - immunology ; Cerebellar Ataxia - metabolism ; Child, Preschool ; Clinical Neurology ; Female ; HEK293 Cells ; Humans ; Life Sciences & Biomedicine ; Lung Neoplasms - complications ; Lung Neoplasms - immunology ; Lung Neoplasms - metabolism ; Neoplasms - complications ; Neoplasms - immunology ; Neoplasms - metabolism ; Neurosciences ; Neurosciences & Neurology ; Paraneoplastic Syndromes, Nervous System - etiology ; Paraneoplastic Syndromes, Nervous System - immunology ; Paraneoplastic Syndromes, Nervous System - metabolism ; Rats ; Receptors, Metabotropic Glutamate - immunology ; Rhabdomyosarcoma - complications ; Rhabdomyosarcoma - immunology ; Rhabdomyosarcoma - metabolism ; Science & Technology</subject><ispartof>Neurology : neuroimmunology & neuroinflammation, 2020-03, Vol.7 (2), p.e658-e658</ispartof><rights>American Academy of Neurology</rights><rights>Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.</rights><rights>Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology. 2019 American Academy of Neurology</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>true</woscitedreferencessubscribed><woscitedreferencescount>28</woscitedreferencescount><woscitedreferencesoriginalsourcerecordid>wos000529951300008</woscitedreferencesoriginalsourcerecordid><citedby>FETCH-LOGICAL-c5190-d617af19f22ce1d600bbb3aa7a03e42897f60f34e012dc9ab73b780c0afd1f5c3</citedby><cites>FETCH-LOGICAL-c5190-d617af19f22ce1d600bbb3aa7a03e42897f60f34e012dc9ab73b780c0afd1f5c3</cites><orcidid>0000-0002-3220-9488 ; 0000-0001-5856-2813 ; 0000-0002-8924-8322 ; 0000-0003-4631-3056 ; 0000-0002-8403-3613</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6943365/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6943365/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,315,728,781,785,865,886,2115,27929,27930,28253,53796,53798</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/31826987$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ruiz-García, Raquel</creatorcontrib><creatorcontrib>Martínez-Hernández, Eugenia</creatorcontrib><creatorcontrib>Joubert, Bastien</creatorcontrib><creatorcontrib>Petit-Pedrol, Mar</creatorcontrib><creatorcontrib>Pajarón-Boix, Elena</creatorcontrib><creatorcontrib>Fernández, Verónica</creatorcontrib><creatorcontrib>Salais, Lidia</creatorcontrib><creatorcontrib>del Pozo, María</creatorcontrib><creatorcontrib>Armangué, Thais</creatorcontrib><creatorcontrib>Sabater, Lidia</creatorcontrib><creatorcontrib>Dalmau, Josep</creatorcontrib><creatorcontrib>Graus, Francesc</creatorcontrib><title>Paraneoplastic cerebellar ataxia and antibodies to metabotropic glutamate receptor 2</title><title>Neurology : neuroimmunology & neuroinflammation</title><addtitle>NEUROL-NEUROIMMUNOL</addtitle><addtitle>Neurol Neuroimmunol Neuroinflamm</addtitle><description>Objective To report the presence of a new neuronal surface antibody against the metabotropic glutamate receptor 2 antibody (mGluR2-Ab) in 2 patients with paraneoplastic cerebellar ataxia. Methods mGluR2-Abs were initially characterized by immunohistochemistry on the rat brain and confirmed by immunofluorescence on HEK293 cells transfected with mGluR2. Additional studies included analysis of potential cross-reactivity with other mGluRs, expression of mGluR2 in patients' tumors, and the effects of mGluR2-Abs on cultures of rat hippocampal neurons. Results Patient 1 was a 78-year-old woman with progressive cerebellar ataxia with an initial relapsing-remitting course who developed a small-cell tumor of unknown origin. Patient 2 was a 3-year-old girl who presented a steroid-responsive acute cerebellitis preceding the diagnosis of an alveolar rhabdomyosarcoma. Patients' serum and CSF showed a characteristic immunostaining of the hippocampus and cerebellum in rat brain sections and immunolabeled the cell surface of live rat hippocampal neurons. HEK293 cells transfected with mGluR1, 2, 3, and 5 confirmed that patients' antibodies only recognized mGluR2. mGluR2-Abs were not detected in 160 controls, 120 with paraneoplastic, autoimmune, or degenerative ataxias, and 40 with autoimmune encephalitis and antibodies against mGluR5 or unknown antigens. Expression of mGluR2 in tumors was confirmed by immunohistochemistry using a commercial mGluR2-Ab. Incubation of live rat hippocampal neurons with CSF of patient 2 did not modify the density of surface mGluR2 clusters. Conclusions mGluR2-Abs are a novel biomarker of paraneoplastic cerebellar ataxia. The potential pathogenic effect of the antibodies is not mediated by downregulation or internalization of neuronal surface mGluR2.</description><subject>Aged</subject><subject>Animals</subject><subject>Autoantibodies - immunology</subject><subject>Autoantibodies - metabolism</subject><subject>Biomarkers, Tumor - immunology</subject><subject>Biomarkers, Tumor - metabolism</subject><subject>Carcinoma, Neuroendocrine - complications</subject><subject>Carcinoma, Neuroendocrine - immunology</subject><subject>Carcinoma, Neuroendocrine - metabolism</subject><subject>Cerebellar Ataxia - etiology</subject><subject>Cerebellar Ataxia - immunology</subject><subject>Cerebellar Ataxia - metabolism</subject><subject>Child, Preschool</subject><subject>Clinical Neurology</subject><subject>Female</subject><subject>HEK293 Cells</subject><subject>Humans</subject><subject>Life Sciences & Biomedicine</subject><subject>Lung Neoplasms - complications</subject><subject>Lung Neoplasms - immunology</subject><subject>Lung Neoplasms - metabolism</subject><subject>Neoplasms - complications</subject><subject>Neoplasms - immunology</subject><subject>Neoplasms - metabolism</subject><subject>Neurosciences</subject><subject>Neurosciences & Neurology</subject><subject>Paraneoplastic Syndromes, Nervous System - etiology</subject><subject>Paraneoplastic Syndromes, Nervous System - immunology</subject><subject>Paraneoplastic Syndromes, Nervous System - metabolism</subject><subject>Rats</subject><subject>Receptors, Metabotropic Glutamate - immunology</subject><subject>Rhabdomyosarcoma - complications</subject><subject>Rhabdomyosarcoma - immunology</subject><subject>Rhabdomyosarcoma - metabolism</subject><subject>Science & Technology</subject><issn>2332-7812</issn><issn>2332-7812</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><sourceid>AOWDO</sourceid><sourceid>EIF</sourceid><recordid>eNqNkl-L1DAUxYMo7rLuNxDpoyBd86dp2hdBhnVdWNSHFXwLN-ntTrRtapI6-u03ZdZx9MlASCC_c3JvTgh5zugF44y__vDl-oIej1o2j8gpF4KXqmH88dH-hJzH-DUzjEupavWUnAjW8Lpt1Cm5_QQBJvTzADE5W1gMaHAYIBSQ4KeDAqYuz-SM7xzGIvlixATGp-DnLLgblgQjJCwCWpyTDwV_Rp70MEQ8f1jPyOd3l7eb9-XNx6vrzdub0krW0rKrmYKetT3nFllXU2qMEQAKqMCKN63qa9qLCnPlnW3BKGFUQy2FvmO9tOKMvNn7zosZsbM4pQCDnoMbIfzSHpz--2RyW33nf-i6rYSoZTZ4-WAQ_PcFY9Kji3ZtPz_JEjUXXArKVFVntNqjNvgYA_aHaxjVayY6Z6L_zSTLXhyXeBD9TiADr_bADo3vo3U4WTxg2UXytpVMrIarXfP_9MYlSM5PG79M6U8DOz8kDPHbsOww6C3CkLY6N9moirKSU07pqi_XH0PFPYoxuYg</recordid><startdate>20200301</startdate><enddate>20200301</enddate><creator>Ruiz-García, Raquel</creator><creator>Martínez-Hernández, Eugenia</creator><creator>Joubert, Bastien</creator><creator>Petit-Pedrol, Mar</creator><creator>Pajarón-Boix, Elena</creator><creator>Fernández, Verónica</creator><creator>Salais, Lidia</creator><creator>del Pozo, María</creator><creator>Armangué, Thais</creator><creator>Sabater, Lidia</creator><creator>Dalmau, Josep</creator><creator>Graus, Francesc</creator><general>American Academy of Neurology</general><general>Lippincott Williams & Wilkins</general><scope>AOWDO</scope><scope>BLEPL</scope><scope>DTL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0002-3220-9488</orcidid><orcidid>https://orcid.org/0000-0001-5856-2813</orcidid><orcidid>https://orcid.org/0000-0002-8924-8322</orcidid><orcidid>https://orcid.org/0000-0003-4631-3056</orcidid><orcidid>https://orcid.org/0000-0002-8403-3613</orcidid></search><sort><creationdate>20200301</creationdate><title>Paraneoplastic cerebellar ataxia and antibodies to metabotropic glutamate receptor 2</title><author>Ruiz-García, Raquel ; Martínez-Hernández, Eugenia ; Joubert, Bastien ; Petit-Pedrol, Mar ; Pajarón-Boix, Elena ; Fernández, Verónica ; Salais, Lidia ; del Pozo, María ; Armangué, Thais ; Sabater, Lidia ; Dalmau, Josep ; Graus, Francesc</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5190-d617af19f22ce1d600bbb3aa7a03e42897f60f34e012dc9ab73b780c0afd1f5c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Aged</topic><topic>Animals</topic><topic>Autoantibodies - immunology</topic><topic>Autoantibodies - metabolism</topic><topic>Biomarkers, Tumor - immunology</topic><topic>Biomarkers, Tumor - metabolism</topic><topic>Carcinoma, Neuroendocrine - complications</topic><topic>Carcinoma, Neuroendocrine - immunology</topic><topic>Carcinoma, Neuroendocrine - metabolism</topic><topic>Cerebellar Ataxia - etiology</topic><topic>Cerebellar Ataxia - immunology</topic><topic>Cerebellar Ataxia - metabolism</topic><topic>Child, Preschool</topic><topic>Clinical Neurology</topic><topic>Female</topic><topic>HEK293 Cells</topic><topic>Humans</topic><topic>Life Sciences & Biomedicine</topic><topic>Lung Neoplasms - complications</topic><topic>Lung Neoplasms - immunology</topic><topic>Lung Neoplasms - metabolism</topic><topic>Neoplasms - complications</topic><topic>Neoplasms - immunology</topic><topic>Neoplasms - metabolism</topic><topic>Neurosciences</topic><topic>Neurosciences & Neurology</topic><topic>Paraneoplastic Syndromes, Nervous System - etiology</topic><topic>Paraneoplastic Syndromes, Nervous System - immunology</topic><topic>Paraneoplastic Syndromes, Nervous System - metabolism</topic><topic>Rats</topic><topic>Receptors, Metabotropic Glutamate - immunology</topic><topic>Rhabdomyosarcoma - complications</topic><topic>Rhabdomyosarcoma - immunology</topic><topic>Rhabdomyosarcoma - metabolism</topic><topic>Science & Technology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ruiz-García, Raquel</creatorcontrib><creatorcontrib>Martínez-Hernández, Eugenia</creatorcontrib><creatorcontrib>Joubert, Bastien</creatorcontrib><creatorcontrib>Petit-Pedrol, Mar</creatorcontrib><creatorcontrib>Pajarón-Boix, Elena</creatorcontrib><creatorcontrib>Fernández, Verónica</creatorcontrib><creatorcontrib>Salais, Lidia</creatorcontrib><creatorcontrib>del Pozo, María</creatorcontrib><creatorcontrib>Armangué, Thais</creatorcontrib><creatorcontrib>Sabater, Lidia</creatorcontrib><creatorcontrib>Dalmau, Josep</creatorcontrib><creatorcontrib>Graus, Francesc</creatorcontrib><collection>Web of Science - Science Citation Index Expanded - 2020</collection><collection>Web of Science Core Collection</collection><collection>Science Citation Index Expanded</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Neurology : neuroimmunology & neuroinflammation</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ruiz-García, Raquel</au><au>Martínez-Hernández, Eugenia</au><au>Joubert, Bastien</au><au>Petit-Pedrol, Mar</au><au>Pajarón-Boix, Elena</au><au>Fernández, Verónica</au><au>Salais, Lidia</au><au>del Pozo, María</au><au>Armangué, Thais</au><au>Sabater, Lidia</au><au>Dalmau, Josep</au><au>Graus, Francesc</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Paraneoplastic cerebellar ataxia and antibodies to metabotropic glutamate receptor 2</atitle><jtitle>Neurology : neuroimmunology & neuroinflammation</jtitle><stitle>NEUROL-NEUROIMMUNOL</stitle><addtitle>Neurol Neuroimmunol Neuroinflamm</addtitle><date>2020-03-01</date><risdate>2020</risdate><volume>7</volume><issue>2</issue><spage>e658</spage><epage>e658</epage><pages>e658-e658</pages><issn>2332-7812</issn><eissn>2332-7812</eissn><abstract>Objective To report the presence of a new neuronal surface antibody against the metabotropic glutamate receptor 2 antibody (mGluR2-Ab) in 2 patients with paraneoplastic cerebellar ataxia. Methods mGluR2-Abs were initially characterized by immunohistochemistry on the rat brain and confirmed by immunofluorescence on HEK293 cells transfected with mGluR2. Additional studies included analysis of potential cross-reactivity with other mGluRs, expression of mGluR2 in patients' tumors, and the effects of mGluR2-Abs on cultures of rat hippocampal neurons. Results Patient 1 was a 78-year-old woman with progressive cerebellar ataxia with an initial relapsing-remitting course who developed a small-cell tumor of unknown origin. Patient 2 was a 3-year-old girl who presented a steroid-responsive acute cerebellitis preceding the diagnosis of an alveolar rhabdomyosarcoma. Patients' serum and CSF showed a characteristic immunostaining of the hippocampus and cerebellum in rat brain sections and immunolabeled the cell surface of live rat hippocampal neurons. HEK293 cells transfected with mGluR1, 2, 3, and 5 confirmed that patients' antibodies only recognized mGluR2. mGluR2-Abs were not detected in 160 controls, 120 with paraneoplastic, autoimmune, or degenerative ataxias, and 40 with autoimmune encephalitis and antibodies against mGluR5 or unknown antigens. Expression of mGluR2 in tumors was confirmed by immunohistochemistry using a commercial mGluR2-Ab. Incubation of live rat hippocampal neurons with CSF of patient 2 did not modify the density of surface mGluR2 clusters. Conclusions mGluR2-Abs are a novel biomarker of paraneoplastic cerebellar ataxia. The potential pathogenic effect of the antibodies is not mediated by downregulation or internalization of neuronal surface mGluR2.</abstract><cop>PHILADELPHIA</cop><pub>American Academy of Neurology</pub><pmid>31826987</pmid><doi>10.1212/NXI.0000000000000658</doi><tpages>5</tpages><orcidid>https://orcid.org/0000-0002-3220-9488</orcidid><orcidid>https://orcid.org/0000-0001-5856-2813</orcidid><orcidid>https://orcid.org/0000-0002-8924-8322</orcidid><orcidid>https://orcid.org/0000-0003-4631-3056</orcidid><orcidid>https://orcid.org/0000-0002-8403-3613</orcidid><oa>free_for_read</oa></addata></record> |
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| subjects | Aged Animals Autoantibodies - immunology Autoantibodies - metabolism Biomarkers, Tumor - immunology Biomarkers, Tumor - metabolism Carcinoma, Neuroendocrine - complications Carcinoma, Neuroendocrine - immunology Carcinoma, Neuroendocrine - metabolism Cerebellar Ataxia - etiology Cerebellar Ataxia - immunology Cerebellar Ataxia - metabolism Child, Preschool Clinical Neurology Female HEK293 Cells Humans Life Sciences & Biomedicine Lung Neoplasms - complications Lung Neoplasms - immunology Lung Neoplasms - metabolism Neoplasms - complications Neoplasms - immunology Neoplasms - metabolism Neurosciences Neurosciences & Neurology Paraneoplastic Syndromes, Nervous System - etiology Paraneoplastic Syndromes, Nervous System - immunology Paraneoplastic Syndromes, Nervous System - metabolism Rats Receptors, Metabotropic Glutamate - immunology Rhabdomyosarcoma - complications Rhabdomyosarcoma - immunology Rhabdomyosarcoma - metabolism Science & Technology |
| title | Paraneoplastic cerebellar ataxia and antibodies to metabotropic glutamate receptor 2 |
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