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[What should we call this group of disease? (author's transl)]
Insights
This study addresses the naming of a cerebrovascular condition characterized by abnormal vascular networks. Researchers propose "MOYAMOYA" as the preferred, unified term for this condition, simplifying international communication.
Area of Science:
- Neurology and Neurosurgery
- Vascular Neurology
- Medical Terminology
Context:
- Multiple names have been used for a specific cerebrovascular condition in Japan, including abnormal vascular networks and spontaneous occlusion of the Circle of Willis.
- This condition is angiographically characterized by internal carotid artery narrowing and an abnormal vascular network of perforating arteries.
- Accurate clinical diagnosis is increasingly possible, yet the condition lacks a definitive clinicopathological entity due to its unknown etiology.
Purpose:
- To investigate the historical naming conventions and current usage of terms for a specific cerebrovascular disorder.
- To determine a consensus among researchers regarding a single, appropriate name for this condition.
- To analyze the advantages and disadvantages of various proposed names, including 'Moyamoya'.
Summary:
- A survey of 306 investigators revealed a consensus for a single name, with a preference for terms describing the abnormal vascular network.
- The name 'Moyamoya' was favored for its brevity, unique meaning in Japanese ('unknown origin'), and global popularity, despite some academic criticisms.
- The study advocates for the simple designation 'MOYAMOYA' over 'Moyamoya disease' to represent this distinct clinical entity.
Impact:
- Establishes a unified and internationally recognizable term ('MOYAMOYA') for a specific cerebrovascular condition.
- Facilitates clearer communication and reduces ambiguity in clinical reporting and research.
- Promotes a more cohesive understanding of this disorder within the global medical community.
Abstract:
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".