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• W2786975656 abstract "Objective: To report the clinical features and outcome of 10 patients with anti-mGluR5 encephalitis and the effects of the antibodies on cultured neurons. Background: Antibodies to mGluR5 have been reported in patients with autoimmune encephalitis and Hodgkin’s lymphoma (Ophelia’s syndrome). Design/Methods: Clinical information was provided by the treating physicians. Antibodies were determined using brain immunohistochemistry and cell-based assays; antibody effects were determined in hippocampal neuronal cultures. Results: We identified 10 patients (January 2006–October 2016): median age 29 years (range 6–75), 4/10 females. The main clinical manifestations were psychiatric (9/10), cognitive (8/10), movement disorders (7/10), seizures (6/10), altered consciousness (4/10), and sleep dysfunction (5/8). Median mRS at disease peak was 4.5; 5/10 patients required intensive care. Six patients had a tumor: 5 Hodgkin’s lymphoma, 1 small cell lung cancer; the latter had progressive ophthalmoplegia, cognitive changes, gait ataxia and SOX1 antibodies. Of the 6 patients with tumor, 3 received immunotherapy and cancer treatment, and 3 cancer treatment only. The 4 patients without tumor received immunotherapy. At last follow-up (median 30 months) 9/10 patients had complete (6) or partial (3) recovery; 1 patient died. One patient with Hodgkin’s lymphoma had a neurological relapse 2.5 years after the first episode of encephalitis. Ancillary tests showed: CSF pleocytosis in 10/10 (median WBC 22) patients; oligoclonal bands in 4/7; MRI abnormalities in 5/10 involving temporal (2/5) or extra-temporal regions (3/5), and EEG abnormalities in 5/7. Antibodies were identified in serum and CSF of all paired samples (5/10). The effects of the antibodies in neuronal cultures will be presented. Conclusions: Anti-mGluR5 encephalitis is characterized by limbic and extra-limbic manifestations and can occur in the absence of a tumor. Patients with tumors other than Hodgkin’s lymphoma may develop an atypical clinical syndrome. Patients respond to immunotherapy (and cancer treatment), but relapses can occur. Disclosure: Dr. Spatolas has received research support from Pierre Mercier Foundation for Science and the Societe Acadmique Vaudoise. Dr. Sabater has nothing to disclose. Dr. Martinez-Hernandez has nothing to disclose. Dr. Rosenfeld has received personal compensation from Athena Diagnostics and Euroimmun. Dr. Graus has nothing to disclose. Dr. Dalmau receives royalties from the editorial board of Up-To-Date. Dr. Dalmau has received royalties from a patent from Memorial Sloan-Kettering Cancer Center and royalty payments and/or for technology and invention on NMDAR, GABA(B), GABAaR, DPPX, and IgLON5 antibody test. Dr. Dalmau has received research support from Euroimmun." @default.
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• W2786975656 date "2017-04-18" @default.
• W2786975656 modified "2023-10-07" @default.
• W2786975656 title "Novel findings in autoimmune encephalitis with antibodies against the metabotropic glutamate receptor 5 (mGluR5) (P1.333)" @default.
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