[What should we call this group of disease? (author's transl)]. 1976

Y Maki

In past several years in Japan, there were many reports on a certain group of cases which were called by many names such as abnormal vascular network in the cerebral basal region, spontaneous occlusion of the circle of Willis, Cerebrovascular Moyamoya disease, cerebral juxabasal telangiectasia and so on. The characteristics of this group are; 1. Angiographically, they show a narrowing at the distal portion of the internal carotid artery, horizontal portions of the anterior and/or middle cerebral arteries with extensively developed perforators, i.e. abnormal vascular network. 2. Nowadays, it is becoming possible to give accurate diagnosis by only clinical information. 3. The cause is not known and so this group has not yet been recognized as a clinico-pathological entity. This group of the cases was first recognized and mainly investigated by Japanese neurosurgeons. But they have used many names as shown Table 1. Such a variety of the names seems to be troublesome for investigator and a single appropriate name should be used. From the Japanese and other country's reports, the name of this disorder can be divided into 5 categories as follows; Category 1: from the viewpoint of clinical symptoms and sign. Category 2: from the viewpoint of only abnormal vascular network on angiograms. Category 3: from the finding of the arterial narrowing. Category 4: from the both findings of the abnormal vascular network and arterial narrowing. Category 5: miscellaneous. The annual changes in usage frequency of various designation about this disorder has investigated and it disclosed the difference between Japan and other countries. The questionairre about the naming was sent to 306 investigators who had reported the cases. And the summary of their response as follow: 1) Most of them agree with determination of a single name. 2) Their most favorite name belongs to Category 2. This means that the most impressive finding was not the obstruction but the vascular network shown in angiograms. 3) The name "Abnormal vascular network" and "Moyamoya" mean the same phenomenon. In spite of having many disputation such as unacademic, not including the idea of arterial obstruction, and difficulty to understand for foreigners, but the name "Moyamoya" was pointed out by many investigators that it has many advantages such as being short and humorous, Moyamoya also has a meaning of unknown origin in Japanese, and already very popular worldwide. In my opinion, this group must not be called as "Moyamoya disease", but simply "MOYAMOYA".

UI MeSH Term Description Entries
D009626 Terminology as Topic Works about the terms, expressions, designations, or symbols used in a particular science, discipline, or specialized subject area. Etymology,Nomenclature as Topic,Etymologies
D002538 Intracranial Arteriovenous Malformations Congenital vascular anomalies in the brain characterized by direct communication between an artery and a vein without passing through the CAPILLARIES. The locations and size of the shunts determine the symptoms including HEADACHES; SEIZURES; STROKE; INTRACRANIAL HEMORRHAGES; mass effect; and vascular steal effect. Arteriovenous Malformations, Cerebral,Intracranial Arteriovenous Malformations, Congenital,AVM (Arteriovenous Malformation) Intracranial,Cerebral Arteriovenous Malformations,Congenital Intracranial Arteriovenous Malformations,Intracranial Arteriovenous Malformation, Ruptured,Ruptured Intracranial Arteriovenous Malformation,Arteriovenous Malformation, Cerebral,Arteriovenous Malformation, Intracranial,Arteriovenous Malformations, Intracranial,Cerebral Arteriovenous Malformation,Intracranial Arteriovenous Malformation,Malformation, Cerebral Arteriovenous,Malformation, Intracranial Arteriovenous,Malformations, Cerebral Arteriovenous,Malformations, Intracranial Arteriovenous
D002561 Cerebrovascular Disorders A spectrum of pathological conditions of impaired blood flow in the brain. They can involve vessels (ARTERIES or VEINS) in the CEREBRUM, the CEREBELLUM, and the BRAIN STEM. Major categories include INTRACRANIAL ARTERIOVENOUS MALFORMATIONS; BRAIN ISCHEMIA; CEREBRAL HEMORRHAGE; and others. Brain Vascular Disorders,Intracranial Vascular Disorders,Vascular Diseases, Intracranial,Cerebrovascular Diseases,Cerebrovascular Insufficiency,Cerebrovascular Occlusion,Brain Vascular Disorder,Cerebrovascular Disease,Cerebrovascular Disorder,Cerebrovascular Insufficiencies,Cerebrovascular Occlusions,Disease, Cerebrovascular,Diseases, Cerebrovascular,Insufficiencies, Cerebrovascular,Insufficiency, Cerebrovascular,Intracranial Vascular Disease,Intracranial Vascular Diseases,Intracranial Vascular Disorder,Occlusion, Cerebrovascular,Occlusions, Cerebrovascular,Vascular Disease, Intracranial,Vascular Disorder, Brain,Vascular Disorder, Intracranial,Vascular Disorders, Brain,Vascular Disorders, Intracranial
D002941 Circle of Willis A polygonal anastomosis at the base of the brain formed by the internal carotid (CAROTID ARTERY, INTERNAL), proximal parts of the anterior, middle, and posterior cerebral arteries (ANTERIOR CEREBRAL ARTERY; MIDDLE CEREBRAL ARTERY; POSTERIOR CEREBRAL ARTERY), the anterior communicating artery and the posterior communicating arteries. Cerebral Arterial Circle,Willis' Circle,Arterial Circle, Cerebral,Circle, Willis',Willis Circle
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001157 Arterial Occlusive Diseases Pathological processes which result in the partial or complete obstruction of ARTERIES. They are characterized by greatly reduced or absence of blood flow through these vessels. They are also known as arterial insufficiency. Arterial Obstructive Diseases,Arterial Occlusion,Arterial Obstructive Disease,Arterial Occlusions,Arterial Occlusive Disease,Disease, Arterial Obstructive,Disease, Arterial Occlusive,Obstructive Disease, Arterial,Occlusion, Arterial,Occlusive Disease, Arterial
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes

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