Abstract

An epidemiological and clinical study of multiple sclerosis (MS) in the Orkney and Shetland Islands showed that the prevalence rate of the disease is the highest in the world (309 and 184/100 000, respectively). The clinical entity, MS, is similar to that found in other parts of the world, except that optic neuritis not followed by MS is rare. Analysis of death certificates indicated that MS has probably occurred at the same rate in these islands for nearly a century. Although the incidence of MS is high, the incidence rate has remained constant over time. A rapidly increasing prevalence of MS has occurred in Orkney, with a more modest increase in Shetland, over the past 20 years, which is largely due to an increase in survival. Demographic factors, case ascertainment, and emigration have contributed little to the increasing prevalence of MS in these islands.