Prevalence, Incidence, and Mortality of Myasthenia Gravis and Myasthenic Syndromes: A Systematic Review

Introduction: No systematic reviews were published in the last years investigating epidemiological data, involving myasthenia gravis (MG) and related myasthenic syndromes. This systematic review aimed to estimate the prevalence, incidence, and mortality of all MG types and myasthenic syndromes worldwide. Methods: All literature published up to February 2024 was retrieved by searching the databases "Medline," "Embase," "ISI Web of Science" and "CINAHL" using the following search terms: (epidemiolog*OR frequency OR prevalence OR incidence OR mortality) AND (myasth*OR "anti-acetylcholine receptor anti body" OR "AChR" OR "MuSK" OR "anti-muscle specific kinase antibody"OR"LRP4"OR"seronegative MG").Results: A total of 94 studies, performed between 1952and 2022, were included. Prevalence of MG ranged from20 to 475 cases per million, with a mean prevalence of173.3 (95% confidence interval [CI]: 129.7-215.5) cases per million and a median prevalence of 129.6 cases per mil-lion. Incidence rates ranged from 2.3 to 61.3 cases per million person-years, with a mean incidence of 15.7 (95%CI: 11.5-19.9) and a median of 13.3 cases. Mortality rates showed a mean of 1.4 (95% CI: 0.8-2.1) cases per million person-years. Acetylcholine receptor (AChR)-MG was the clinical subtype more frequent in terms of prevalence and incidence. Discussion: The prevalence and incidence of MG have significantly increased over the last years worldwide, probably due to the improvement of epidemiological methodologies and current advances in diagnosis. However, we observed a significant variation infrequencies of MG between and within countries because