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This study was supported in part by Instituto Carlos III/FEDER (FIS 17/00234, J.D.) and CIBERER No. CB15/00010, J.D.; NIH RO1NS077851, J.D.; Neuroscience Research Training Scholarship by the American Academy of Neurology, M.S.; Fondation pour la Recherche Medicale (SPF201909009269, France), M.P.P.; Basque Government Doctoral Fellowship Program (PRE_2019_1_0255), E.M.; BETPSY project, a public grant overseen by the French National Research Agency (ANR), as part of the second Investissements d ' Avenir program (ANR-18-RHUS-0012), J.H.; Jubilaumsfonds der Osterreichischen Nationalbank (project 16919), R.H.; Safra Foundation, and Fundacio CELLEX, J.D.

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Clinical features, prognostic factors, and antibody effects in anti-mGluR1 encephalitis

Publicado en:Neurology. 95 (22): E3012-E3025 - 2020-12-01 95(22), DOI: 10.1212/wnl.0000000000010854

Autores: Spatola, Marianna; Pedrol, Mar Petit; Maudes, Estibaliz; Simabukuro, Mateus; Muniz-Castrillo, Sergio; Pinto, Anne-Laurie; Wandinger, Klaus-Peter; Spiegler, Juliane; Schramm, Peter; Dutra, Livia Almeida; Iorio, Raffaele; Kornblum, Cornelia; Bien, Christian G; Hoeftberger, Romana; Leypoldt, Frank; Titulaer, Maarten J; Smitt, Peter Sillevis; Honnorat, Jerome; Rosenfeld, Myrna R; Graus, Francesc; Dalmau, Josep

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Resumen

To clinically characterize patients with anti-metabotropic glutamate receptor (mGluR)1 encephalitis, identify prognostic factors, and study the IgG subclasses and effects of antibodies on neuronal mGluR1 clusters.Clinical information of new and previously reported patients was reviewed. Antibodies to mGluR1 and IgG subclasses were determined with brain immunohistochemistry and cell-based assays, and their effects on mGluR1 clusters were studied on rat hippocampal neurons.Eleven new patients were identified (10 adults, 1 child), 4 females. In these and 19 previously reported cases (n=30, median age 55) the main clinical manifestation was a subacute cerebellar syndrome that in 25 (86%) patients was associated with behavioral/cognitive changes or other neurological symptoms. A tumor was found in 3/26 (11%). Brain MRI was abnormal in 7/19 (37%) at onset and showed cerebellar atrophy in 10/12 (83%) at follow-up. Twenty-five/30 (83%) patients received immunotherapy. Follow-up was available from 25: 13 (52%) had clinical stabilization, 10 (40%) significant improvement, and 2 died. At the peak of the disease, patients with bad outcome (mRS>2, n=7) were more likely to have higher degree of initial disability, as reflected by a worse SARA (Scale for Assessment and Rating of Ataxia) score, and the more frequent need of assistance to walk. Antibodies to mGluR1 were mainly IgG1 and caused a significant decrease of mGluR1 clusters in cultured neurons.Anti-mGluR1 encephalitis manifests as a severe cerebellar syndrome, often resulting in long-term disability and cerebellar atrophy. The antibodies are pathogenic and cause significant decrease of mGluR1 clusters in cultured neurons.© 2020 American Academy of Neurology.

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