Background: Seizure commonly presents as an initial symptom and plays an important role in the clinical presentation and quality of life of patients with low-grade glioma (LGG). To date, the mechanism and genetic alterations underlying tumor-related seizures in LGG remain to be fully elucidated. Both isocitrate dehydrogenase 1/2 (IDH1/2) mutation and seizure frequently occur in patients with LGG. We set out to investigate the potential relationship between IDH1/2 mutation and presentation of seizure preoperatively, and observe whether or not IDH1/2 mutation influences seizure control postoperatively. Methods: A total of 311 adult patients with LGG were enrolled in our study with both clinical data and IDH1/2 mutation data available. IDH1/2 mutation was detected directly by pyro-sequencing. The chi-squared test was performed to determine whether the IDH1/2 mutation has any relevance to seizure onset and to evaluate the potential impact that IDH1/2 mutation may exert on seizure control postoperatively. Results: Seizure presented as an initial symptom in 71.4% (222/311) of patients with LGG, among which 189 patients were detected to bear IDH1/2 mutation in their tumors (P=0.035, chi-squared test). However, IDH1/2 mutation does not seem to contribute to the seizure control postoperatively (P = 0.350 and 0.577 for the 6- and 12-month follow-up, respectively, chi-squared test). Conclusions: IDH1/2 mutation occurs more frequently in LGG patients with seizure as an initial symptom, suggesting a potential relationship between this genetic phenotype and clinical seizure presentation. IDH1/2 mutation shows no prognostic value for postoperative seizure control. (C) 2014 Published by Elsevier B.V.