Progression of Moyamoya Disease


Moyamoya disease is characterized by progressive stenosis of the terminal portion of the internal carotid artery (ICA) and its main branches [1]. The disease associated with the development of dilated, fragile collateral vessels at the base of the brain, which are termed moyamoya vessels [1]. Regardless of the course, moyamoya disease inevitably progresses in the majority of patients.


Internal Carotid Artery Hepatocyte Growth Factor Magnetic Resonance Angiography Anterior Cerebral Artery Moyamoya Disease 
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 “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288–299PubMedCrossRefGoogle Scholar
  2. 2.
    Suzuki J, Kodama N (1983) Moyamoya disease-a review. Stroke 14:104–109PubMedCrossRefGoogle Scholar
  3. 3.
    Houkin K, Yoshimoto T, Kuroda S et al (1996) Angiographic analysis of moyamoya disease-how does moyamoya disease progress? Neurol Med Chir (Tokyo) 36:783–788CrossRefGoogle Scholar
  4. 4.
    Kim SK, Seol HJ, Cho BK et al (2004) Moyamoya disease among young patients: its aggressive clinical course and the role of active surgical treatment. Neurosurgery 54:840–846PubMedCrossRefGoogle Scholar
  5. 5.
    Ezura M, Yoshimoto T, Fujiwara S et al (1995) Clinical and angiographic follow-up of childhood-onset moyamoya disease. Childs Nerv Syst 11:591–594PubMedCrossRefGoogle Scholar
  6. 6.
    Takahashi A, Fujiwara S, Suzuki J (1986) Long-term follow-up angiography of moyamoya disease-cases followed from childhood to adolescence. No Shinkei Geka 14:23–29PubMedGoogle Scholar
  7. 7.
    Tomida M, Muraki M, Yamasaki K (2000) Angiographically verified progression of moyamoya disease in an adult. Case report. J Neurosurg 93:1055–1057PubMedCrossRefGoogle Scholar
  8. 8.
    Kuroda S, Ishikawa T, Houkin K et al (2005) Incidence and clinical features of disease progression in adult moyamoya disease. Stroke 36:2148–2153PubMedCrossRefGoogle Scholar
  9. 9.
    Narisawa A, Fujimura M, Tominaga T (2009) Efficacy of the revascularization surgery for adult-onset moyamoya disease with the progression of cerebrovascular lesions. Clin Neurol Neurosurg 111:123–126PubMedCrossRefGoogle Scholar
  10. 10.
    Fukui M (1997) Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (‘moyamoya’ disease). Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan. Clin Neurol Neurosurg 2:238–240Google Scholar
  11. 11.
    Kawano T, Fukui M, Hashimoto N et al (1994) Follow-up study of patients with “unilateral” moyamoya disease. Neurol Med Chir (Tokyo) 34:744–747CrossRefGoogle Scholar
  12. 12.
    Kurose K, Kishi H, Nishijima Y (1991) Moyamoya disease developing from bilateral moyamoya disease. Neurol Med Chir (Tokyo) 31:597–599CrossRefGoogle Scholar
  13. 13.
    Matsushima T, Take S, Fujii K et al (1988) A case of moyamoya disease with progressive involvement from unilateral to bilateral. Surg Neurol 30:471–475PubMedCrossRefGoogle Scholar
  14. 14.
    Seol HJ, Wang KC, Kim SK et al (2006) Unilateral (probable) moyamoya disease: long-term follow-up of seven cases. Childs Nerv Syst 22:145–150PubMedCrossRefGoogle Scholar
  15. 15.
    Kelly ME, Bell-Stephens TE, Marks MP et al (2006) Progression of unilateral moyamoya disease: a clinical series. Cerebrovasc Dis 22:109–115PubMedCrossRefGoogle Scholar
  16. 16.
    Kagawa R, Okada Y, Moritake K et al (2004) Magnetic resonance angiography demonstrating adult moyamoya disease progressing from unilateral to bilateral involvement. Neurol Med Chir (Tokyo) 44:183–186CrossRefGoogle Scholar
  17. 17.
    Murphy MJ (1980) Progressive vascular changes in moyamoya syndrome. Stroke 11:656–658PubMedCrossRefGoogle Scholar
  18. 18.
    Wanifuchi H, Takeshita M, Aoki N (1996) Adult moyamoya disease progressing from unilateral to bilateral involvement. Neurol Med Chir (Tokyo) 36:87–90CrossRefGoogle Scholar
  19. 19.
    Smith ER, Scott RM (2008) Progression of disease in unilateral moyamoya syndrome. Neurosurg Focus 24:E17PubMedCrossRefGoogle Scholar
  20. 20.
    Houkin K, Abe H, Yoshimoto T et al (1996) Is “unilateral” moyamoya disease different from moyamoya disease? J Neurosurg 85:772–776PubMedCrossRefGoogle Scholar
  21. 21.
    Kuroda S, Houkin K (2008) Moyamoya disease: current concepts and future perspectives. Lancet Neurol 7:1056–1066PubMedCrossRefGoogle Scholar
  22. 22.
    Scott RM, Smith ER (2009) Moyamoya disease and moyamoya syndrome. N Engl J Med 360:1226–1237PubMedCrossRefGoogle Scholar
  23. 23.
    Takagi Y, Kikuta K, Nozaki K et al (2007) Expression of hypoxia-inducing factor-1 alpha and endoglin in intimal hyperplasia of the middle cerebral artery of patients with Moyamoya disease. Neurosurgery 60:338–345PubMedCrossRefGoogle Scholar
  24. 24.
    Kawaguchi S, Sakaki T, Morimoto T et al (1996) Characteristics of intracranial aneurysms associated with moyamoya disease. A review of 111 cases. Acta Neurochir (Wien) 138:1287–1294CrossRefGoogle Scholar
  25. 25.
    Morioka M, Hamada J, Todaka T et al (2003) High-risk age for rebleeding in patients with hemorrhagic moyamoya disease: long-term follow-up study. Neurosurgery 52:1049–1055PubMedCrossRefGoogle Scholar
  26. 26.
    Yamada M, Fujii K, Fukui M (2005) Clinical features and outcomes in patients with asymptomatic moyamoya disease — from the results of nation-wide questionnaire survey. No Shinkei Geka 33:337–342PubMedGoogle Scholar
  27. 27.
    Kuroda S, Ishikawa T, Houkin K et al (2002) Clinical significance of posterior cerebral artery stenosis/occlusion in moyamoya disease. No Shinkei Geka 30:1295–300PubMedGoogle Scholar
  28. 28.
    Honda M, Ezaki Y, Kitagawa N et al (2006) Quantification of the regional cerebral blood flow and vascular reserve in moyamoya disease using split-dose iodoamphetamine I 123 single-photon emission computed tomography. Surg Neurol 66:155–159PubMedCrossRefGoogle Scholar
  29. 29.
    Honda M, Kitagawa N, Tsutsumi K et al (2005) Magnetic resonance angiography evaluation of external carotid artery tributaries in moyamoya disease. Surg Neurol 64:325–330PubMedCrossRefGoogle Scholar
  30. 30.
    Houkin K, Nakayama N, Kuroda S et al (2004) How does angiogenesis develop in pediatric moyamoya disease after surgery? A prospective study with MR angiography. Childs Nerv Syst 20:734–741PubMedGoogle Scholar

Copyright information

© Springer 2010

Authors and Affiliations

  1. 1.Department of NeurosurgeryNagasaki University School of MedicineNagasaki-cityJapan

Personalised recommendations