Pathology of Moyamoya Disease

  • Kent Doi
  • Ken-ichiro Kikuta


Moyamoya disease (MMD) is a cerebrovascular occlusive disease first reported by Japanese surgeons in 1957 as hypoplasia of the bilateral internal carotid arteries [1]. The entity is characterized by steno-occlusive changes at the terminal portion of the bilateral internal carotid arteries and by development of an abnormal vascular network near the arterial occlusion [2, 3].

In the criteria prepared by the Research Committee in Japan [4], instructive pathological findings are as follows: (1) intimal thickening and the resulting stenosis or occlusion of the lumen is observed in and around the terminal portion of the internal carotid artery, usually on both sides; lipid deposits are occasionally seen in the proliferating intima; (2) arteries constituting the circle of Willis, such as the anterior and the middle cerebral and the posterior communicating arteries, often show stenosis of various degrees or occlusion associated with fibrocellular thickening of the intima, waving of the internal elastic lamina, and attenuation of the media; (3) numerous small vascular channels (perforators and anastomotic branches) are observed around the circle of Willis; and (4) reticular conglomerates of small vessels are often seen in the pia mater.


Down Syndrome Moyamoya Disease Internal Elastic Lamina Moyamoya Syndrome Bilateral Internal Carotid Artery 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



Antiphospholipid antibody syndromes




Endothelial progenitor cell




Middle cerebral artery


Moyamoya disease


Magnetic resonance


Messenger ribonucleic acid


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© Springer 2010

Authors and Affiliations

  1. 1.Department of NeurosurgeryKyoto University Graduate School of Medicine, Yoshida-Konoe-choSakyo-kuJapan
  2. 2.Division of Neurosurgery, Department of Sensory and Locomotor Medicine, Faculty of Medical SciencesUniversity of FukuiYoshida-gunJapan

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