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

Marianna Spatola, Mar Petit Pedrol, Estibaliz Maudes, Mateus Simabukuro, Sergio Muñiz-Castrillo, Anne Laurie Pinto, Klaus Peter Wandinger, Juliane Spiegler, Peter Schramm, Lívia Almeida Dutra, Raffaele Iorio, Cornelia Kornblum, Christian G. Bien, Romana Höftberger, Frank Leypoldt, Maarten J. Titulaer, Peter Sillevis Smitt, Jérôme Honnorat, Myrna R. Rosenfeld, Francesc GrausJosep Dalmau


OBJECTIVE: To clinically characterize patients with anti-metabotropic glutamate receptor (mGluR) 1 encephalitis, to identify prognostic factors, and to study the immunoglobulin G (IgG) subclasses and effects of antibodies on neuronal mGluR1 clusters. METHODS: Clinical information on 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. RESULTS: Eleven new patients were identified (10 adults, 1 child);4 were female. In these and 19 previously reported cases (n = 30, median age 55 years), the main clinical manifestation was a subacute cerebellar syndrome that in 25 (86%) patients was associated with behavioral/cognitive changes or other neurologic symptoms. A tumor was found in 3 of 26 (11%). Brain MRI was abnormal in 7 of 19 (37%) at onset and showed cerebellar atrophy in 10 of 12 (83%) at follow-up. Twenty-five of 30 (83%) patients received immunotherapy. Follow-up was available for 25: 13 (52%) had clinical stabilization; 10 (40%) showed significant improvement; and 2 died. At the peak of the disease, patients with bad outcome at 2 years (modified Rankin Scale score > 2, n = 7) were more likely to have higher degree of initial disability, as reflected by a worse Scale for Assessment and Rating of Ataxia score, and more frequent need of assistance to walk. Antibodies to mGluR1 were mainly IgG1 and caused a significant decrease of mGluR1 clusters in cultured neurons. CONCLUSIONS: 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.

Original languageEnglish
Issue number22
Pages (from-to)e3012-e3025
Publication statusPublished - 01.12.2020

Research Areas and Centers

  • Academic Focus: Biomedical Engineering


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