Juvenile myasthenia gravis in Norway: A nationwide epidemiological study.

BACKGROUND: The aim of this study was to assess the incidence rate and prevalence of autoimmune myasthenia gravis (MG) among children in Norway. METHODS: This retrospective population-based study was performed in Norway from January 2012 to December 2013. Cases of juvenile MG (JMG) with onset < 18 years were identified through searches in coding systems of electronic patient records at the 15 main hospitals in Norway from 1989 to 2013. In addition, the acetylcholine receptor antibody database at Haukeland University Hospital and the clinical nationwide MG database at Oslo University Hospital were searched for cases of JMG. Diagnosis and age at onset were verified through medical records. Incidence and prevalence rates were calculated using the Norwegian population as reference. RESULTS: In total 63 unique JMG cases were identified. This corresponds to an average annual incidence rate of 1.6 per million. Incidence rate was stable over the study period. Prevalence of JMG was 3.6-13.8 per million. Females constituted the majority of JMG cases (55 vs 8 males). The risk of JMG was higher among females both in the postpubertal and prepubertal group (p < 0.001 and p = 0.02, respectively). CONCLUSION: This study confirms the rarity of JMG in Norway, especially among males, and shows a stable incidence rate over the last 25 years.

Myasthenia gravis epidemiology in a national cohort; combining multiple disease registries.

OBJECTIVES: There is a wide variation in reported prevalence and incidence of myasthenia gravis (MG). In this study, we aimed to evaluate the validity of two nationwide databases by comparing prevalence and incidence rates reported from three recent studies using the two databases as case-finding method. MATERIALS AND METHODS: Two different Norwegian nationwide databases were used: the acetylcholine receptor antibody database (reference cohort) and the Norwegian Prescription Database (NorPD) (study cohort). Presence of acetylcholine receptor antibodies (AChR) is specific for MG. Up to 85% of MG patients are AChR antibody-positive. All samples from the whole country were tested at one laboratory. NorPD contains patient information on all prescriptions of pyridostigmine. RESULTS: Prevalence was 131 per million in the study cohort and 145 per million estimated from the reference cohort (Jan 1, 2008). No significant difference in prevalence between the study cohort and the reference cohort was found (SIR 1.1, 95% CI 1.0-1.2). The annual incidence rate was 16.0 per million in the study cohort and 8.8 per million estimated from the reference cohort, twofold more new MG patients were found in the study cohort compared to estimated figures from the reference cohort (SIR 1.8; 1.4-2.3). CONCLUSIONS: This study confirms an optimal and unbiased case finding in both databases. Our calculated prevalence and incidence rates are in line with previous population-based studies. There was good agreement in prevalence reported from the two databases. The discrepancy in incidence is expected to diminish as years of study are increasing in NorPD.