Objective: To determine the diagnoses in a population of patients with presumed optic neuritis in China and compare the spectrum of etiologies with those in western countries. Methods: We studied a group of 204 patients referred with a presumptive diagnosis of optic neuritis (ON) to a teaching hospital. We conducted detailed medical and family history, thorough ophthalmologic and neurological examinations, and laboratory tests including neuroimaging. Patients were diagnosed using international diagnostic criteria.Results: 113 cases (55.4%) met ON criteria, among which 83 cases (73.5%) were considered as idiopathic demyelinating optic neuritis (IDON). Brain MRI white matter lesions were found in 11 of 78 (14.1%) clinically "isolated" IDON cases. Tuberculosis meningitis accounted for 2 cases and syphilis for 1. The cause of 26 cases (23.0%) remained uncertain, but these cases were still likely to be IDON accompanied by secondary axonal degeneration. Thirty-eight cases (18.6%) were confirmed to be Leber's hereditary optic neuropathy Etiological profile of presumptive optic neuritis in China