History of Disease Entity and Diagnosis Criteria

This chapter reviews the history of this mysterious moyamoya disease. Since the 1950s, this rare disease has been discovered in Japan and gradually gaining recognition around the world. It has been just 50 years since Suzuki and Takaku first published the term “moyamoya disease” in an English language journal in 1969. Looking back over the past 50 years, the author would like to review the state of affairs at the time when the causes and concepts of this disease were hotly debated. It may be helpful to us in the future when new diseases appear in front of us. It is no secret, however, that it is nearly impossible to write a history that will satisfy everyone. Therefore, the author appreciates it if the readers would kindly understand that this historical review is only from the author’s point of view and they would forgive him. In this chapter, the author describes the evolution of the diagnostic criteria for moyamoya disease. Since the diagnostic criteria for moyamoya disease were first established in 1979 by a Japanese research group, they have been revised approximately every 10 years in 1988, 1995, 2009, and 2018. The author would like to look back at that history and explore the background with the readers. Finally, the challenges of the diagnostic criteria and the prospects for the future will be discussed.