Moyamoya disease is a unique cerebrovascular disease with steno-occlusive changes at the terminal portion of the internal carotid artery and fine vascular network, the so-called “moyamoya” vessels [1]. This vascular network forms a collateral pathway and compensates reduced cerebral blood flow due to steno-occlusive changes of the trunk arteries. The characteristics of moyamoya disease can be summarized as follows. Moyamoya disease shows racial difference in incidence and predominantly occurs in the Eastern Asia among a worldwide distribution. Clinical manifestation includes ischemia and hemorrhage, epilepsy, headache, etc., and young patients usually present with ischemia and adult patients with either ischemia or hemorrhage [2]. Evidence indicates that revascularization surgery can prevent an ischemic event, although its effect on prevention of hemorrhagic events is yet to be determined [– 6]. Since the familial occurrence reaches 12%, an intense effort has been focused on genetic analysis during the past decade which has found that several genetic loci associate with this disease [7, 8]. Nevertheless, the conclusive pathogenesis of this disease still remains unknown. In this chapter, the author seeks to focus on the diagnostic criteria, the definition, and the history of this disease.


Internal Carotid Artery Down Syndrome Vascular Change Moyamoya Disease Intimal Layer 
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.


  1. 1.
    Suzuki J, Takaku A (1969) Cerebrovascular “Moaymoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288–299PubMedCrossRefGoogle Scholar
  2. 2.
    Kuroda S, Houkin K (2008) Moyamoya disease: current concepts and future perspectives. Lancet Neurol 7:1056–1066PubMedCrossRefGoogle Scholar
  3. 3.
    Roach ES, Colomb MR, Adam R et al (2008) Management of stroke in infants and children. A scientific statement from a special writing group of the American heart association stroke council and the council on cardiovascular disease in the young. Stroke 39:2644–2691PubMedCrossRefGoogle Scholar
  4. 4.
    Scott RM, Smith ER (2009) Moyamoya disease and moyamoya syndrome. N Eng J Med 360: 1226–1237CrossRefGoogle Scholar
  5. 5.
    Hallemeier CL, Rich KM, Grubb RL et al (2006) Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke 37:1490–1496PubMedCrossRefGoogle Scholar
  6. 6.
    Mesiwala AH, Sviri G, Fatemi N et al (2008) Long-term outcome of superficial temporal artery-middle cerebral artery bypass for patients with moyamoya disease in the U.S. Neurosurg Focus. doi: 10.3171/FOC/2008/24/2/E15Google Scholar
  7. 7.
    Kuriyama S, Kusaka Y, Fujimura M et al (2008) Prevalence and clinicoepidemiological features of moyamoya disease in Japan. Findings from a nationwide epidemiological surgery. Stroke 39:42–47PubMedCrossRefGoogle Scholar
  8. 8.
    Achrol AD, Guzman R, Lee M et al (2009) Pathophysiology and genetic factors in moyamoya disease. Neurosurg Focus. doi: 10.3171.2009.1.FOCUS08302Google Scholar
  9. 9.
    Fukui M, Members of the Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan (1997) Guideline for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (‘Moyamoya’ disease). Clin Neurol Neurosurg 99:S238–S240Google Scholar
  10. 10.
    Kuroda S, Hashimoto N, Yoshimoto T et al (2007) Radiological findings, clinical course, and outcome in asymptomatic moyamoya disease: results of multicenter survey in Japan. Stroke 38:1430–1435PubMedCrossRefGoogle Scholar
  11. 11.
    Smith ER, Scott RM (2008) Progression of disease in unilateral moyamoya syndrome. Neurosurg Focus doi: 10.3171/FOC/2008/24/2/E17Google Scholar
  12. 12.
    Mineharu Y, Liu W, Inoue K, et al (2008) Autosomal dominant moyamoya disease maps to chromosome 17q25.3. Neurology 70:2357–2363PubMedCrossRefGoogle Scholar
  13. 13.
    Ikezaki K, Loftus CM (2001) Quasi-moyamoya disease: definition, classification, and therapy. In Moyamoya disease, Ikezaki K, Loftus CM (eds) AANS, USA, pp 23–41Google Scholar
  14. 14.
    Takeuchi K, Shimizu K (1957) Hypoplasia of the bilateral internal carotid arteries. Brain Nerve (Tokyo) 9:37–43Google Scholar
  15. 15.
    Kudo T, Takayama R, Mikawakuchi K, et al. (1957) Occlusion of internal carotid artery. Brain Nerve (Tokyo) 9:757Google Scholar
  16. 16.
    Weidner W, Hanafee W, Markham CH (1965) Intracranial collateral circulation via leptomeningeal and rete mirabile anastomoses. Neurology 15:39–48PubMedCrossRefGoogle Scholar
  17. 17.
    Kraynbuhl HA, Yasargil MG (1965) Cerebral angiography. Butterworth, LondonGoogle Scholar
  18. 18.
    Leeds NE, Abott KH (1965) Collateral circulation in cerebrovascular disease in childhood via rete mirabile and perforating branches of anterior choroidal and posterior cerebral arteries. Radiology 85:628–634PubMedGoogle Scholar
  19. 19.
    Nishimoto A, Takeuchi T (1968) Abnormal cerebrovascular network related to the internal carotid arteries. J Neurosurg 29:255–260PubMedCrossRefGoogle Scholar
  20. 20.
    Kudo T (1968) Spontaneous occlusion of the circle of Willis: a disease apparently confined to Japanese. Neurology 18:485–496PubMedCrossRefGoogle Scholar
  21. 21.
    Suzuki J (1983) Moyamoya disease. Springer, Tokyo, preface VII–VIIIGoogle Scholar

Copyright information

© Springer 2010

Authors and Affiliations

  1. 1.Department of NeurosurgeryTohoku University Graduate School of MedicineSendaiJapan

Personalised recommendations