Moyamoya Disease

The first mention of moyamoya disease as a distinct disease entity was in a paper I published in 1965. The abnormal net- like vessels at the base of the brain seen in cerebral angio- grams of this disease were described by most native speakers of Japanese as &quote;moyamoya,&quote; a Japanese expression for some- thing hazy, such as a puff of cigarette smoke drifting in the air. In fact, prior to my advocacy of this term, this type of vascular network was often referred to as &quote;moyamoya vessels&quote; by Japanese researchers. In 1969, Dr. A. Takaku and I submitted a paper to the Ar- chives of Neurology entitled &quote;A Disease Showing Abnormal Net-like Vessels at the base of the Brain,&quote; with a subtitle of &quote;Moyamoya Disease.&quote; The editor, however, interchanged the main title and the subtitle and brought this term &quote;moyamoya&quote; to fame! Since then, researchers throughout the world have been using this poetic word! For a nicknaming godfather like me, it is a special joy to have this Japanese word enter the honored ranks ofmedica1 terminology. My first publication concerning this lesion was a report discussing six cases in 1963. Therefore it is now 20 years since I first became aware of and started to study moyamoya dis- ease. At first, I merely thought that it was a strange vascular network, unique to these first few patients, and I reported these cases as being cases of acquired collateral vessels.