Childhood myasthenia gravis in Japan : Pathophysiology and treatment options

Abstract Myasthenia gravis (MG) is a disease that causes muscle weakness and fatigue due to an abnormality in the neuromuscular junction, which prevents the transmission of information. In the 1970s, it became clear that MG was caused by an autoimmune mechanism, and treatments began to be developed. Most studies on the pathophysiology and treatment of MG have been conducted in adults. However, even in terms of acetylcholine receptor antibodies, which are related to MG pathophysiology, adults and children differ significantly. Moreover, epidemiological studies around the world have revealed significant differences in the prevalence of MG between East Asia, Europe, and the United States. Treatment methods also differ slightly across countries, depending on the differences in pathophysiology and medical practices that have been developed in each region of the world. Japanese pediatric neurologists have revealed a pathophysiology unique to children and differences in treatment methods for childhood MG. However, few studies have systematically reported these findings. Therefore, this article reviews the current basic concepts related to the pathophysiology and treatment of childhood MG in Japan.