Moyamoya Disease

  • Ami AttaliEmail author
  • Asma Asif
  • Stephanie Cook


Moyamoya disease (MMD) is a chronic progressive vascular occlusive disease causing bilateral stenosis of the distal internal carotid arteries and anterior and middle cerebral arteries. There is also an abnormal vascular formation of fragile collateral vessels at the base of the brain. See Fig. 103.1. Aneurysms can form in these collateral vessels [1, 2]. Normal cerebral blood vessels may vasodilate in response to chronic ischemia predisposing these patients to ischemia with decreased cerebral perfusion pressure. The term moyamoya derives from Japanese, meaning “haze-like,” a term that describes the puff of smoke angiographic appearance of the cerebral arterial collateral circulation. MMD is most prevalent in the East Asian population, particular in Japan, suggesting a possible genetic etiology. Ten to twelve percentage of patients have a familial predisposition with an autosomal-dominant inheritance [3]. However, MMD is seen less frequently in these populations after relocation to North America and Europe [4]. Patients who demonstrate the classical angiographic appearance of moyamoya, without any other risk factors, are known to have the disease, while those with any of the associated conditions are said to have the syndrome [5].


Moyamoya disease Pregnancy and MMD Vascular occlusive Japan Eccentric fibrocellular thickening Arterial stenosis Microaneurysm Growth factors – HIF-1 VEGF bFGF Transforming growth factor-B1 Matrix metalloproteinase Caspase-3-mediated apoptosis Intracranial hemorrhage Ischemic stroke Recurrent transient ischemic attacks Arteriovenous malformations Reticulin fibers Preeclampsia LMWH Nifedipine Magnesium sulfate Cerebral perfusion pressure 


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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Anesthesiology, Wayne State University (WSU) School of MedicineHenry Ford HospitalDetroitUSA
  2. 2.Department of AnesthesiologyHenry Ford HospitalDetroitUSA

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