Immunological aspects of moyamoya disease

Abstract

Immunological phenomena have pervasive effects on the human body. Many physiological and pathological processes involve the immune system. Because of this pervasiveness, it is often difficult to know whether an observed immunological reaction truly underlies a process or whether it is simply an epiphenomenon. The role of the immune system in moyamoya disease is a controversial issue. The etiology of moyamoya disease is currently unknown. Various etiologies, genetic and environmental, have been proposed; however, none have been definitively proven. There is a lot of evidence for immunological abnormalities in patients with moyamoya disease. However, much of the evidence is indirect and lacks a solid causal relationship. Nonetheless, it is strongly believed that immunological processes play an important role at least in the progression of moyamoya disease. In this chapter, the pathological and epidemiological evidence that supports the immunological origin of moyamoya disease is discussed. © 2010 Springer-Verlag Tokyo.