Pathophysiology of Moyamoya Disease

  • Seung-Ki KimEmail author
  • Ji Yeoun Lee
  • Kyu-Chang Wang
Part of the Stroke Revisited book series (STROREV)


Moyamoya disease (MMD) is a cerebrovascular disease that is caused by idiopathic occlusion of the major intracranial arteries. The essential pathologic feature of the occluded artery is intimal thickening, with smooth muscle cell invasion and attenuation of the media. It is one of the most common causes of stroke in children, and it has a bimodal age peak in prevalence at mid-childhood and in a patient’s 40s. Pediatric patients usually present with transient ischemic attacks (TIAs), and indirect revascularization procedures show good postoperative outcome. Although hemorrhage is very rarely seen in children, it is not as uncommon in the adult population. For adults, direct revascularization operations predominate as the treatment modality. Studies to elucidate the pathophysiology of MMD have been performed, focusing on systemic factors that can cause vessel changes, such as cytokines, circulating cells, and autoimmunity. Additionally, biomechanical evaluations have been performed to explain the focal nature of the disease. Recent advancements in genomics have also revealed candidate genes to explain the pathophysiology.


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© Springer Science+Business Media Singapore 2018

Authors and Affiliations

  1. 1.Department of NeurosurgerySeoul National University Hospital, Seoul National University College of MedicineSeoulSouth Korea
  2. 2.Department of AnatomySeoul National University College of MedicineSeoulSouth Korea
  3. 3.Department of NeurosurgerySeoul National University HospitalSeoulSouth Korea

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