Objective To explore the clinical manifestations,treatment and prognosis of encephalitis with metabotropic glutamate receptors 1(mGluR1)antibody in order to improve clinicians'understanding of this disease.Methods The clinical data of a patient with mGluR1 antibody was reported,and a retrospective analysis was carried out based on 34 patients with mGluR1 antibody in the literature.Results A 38-year-old man mainly manifested as dizzy and ataxia for about 2 months.The cerebrospinal fluid(CSF)protein and IgG index were slightly elevated,while head MRI scan and enhanced examination showed no criminal lesions.The serum and CSF mGluR1 IgG titers were both 1∶100.Diagnosis was made as anti-mGluR1 antibody related encephalitis.Symptoms were significantly relieved after methylprednisolone pulse,intravenous immunoglobulin and rituximab therapy.Including our case,35 cases have been reported.The average age of these patients was(48.3+20.3)years old.The main clinical symptoms included ataxia(33 cases)and decreased cognitive function(11 cases).Eight patients(22.9％)were concomitant with tumor,and all patients'serum and/or cerebrospinal fluid anti-mGluR1 antibodies was positive.85.7％(30/35)of the patients received immunotherapy,of which 11 patients received 2 immunotherapies,and 11 patients received 3 or more immunotherapies.At the end of follow-up,21 patients(60.0％)improved,4 patients(11.4％)relapsed,7 patients(20.0％)remained stable,1 patient(5.7％)remained unchanged and 1 patient(2.9％)died.Conclusions Anti-mGluR1antibody related encephalitis mainly manifests cerebellar ataxia.Serum or CSF anti-mGluR1 antibody testing is helpful for diagnosis.Most patients improve after treated with glucocorticoids combined with immunosuppressants or rituximab,while a small number of patients have recurrence and poor prognosis.
维普
万方数据