Future Perspectives in Moyamoya Disease

  • Byung-Kyu Cho


It has been half a century since K. Takeuchi and K. Shimizu first reported a new entity in vascular disease in 1957, moyamoya disease (MMD) [1]. This disease is a relatively rare vascular disease and has a high prevalence in children and Asian ethnics; thus, it has not been of great interest to physicians and researchers in non-Asian countries. The Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare in Japan launched a project in 1977 to investigate the disease, and continues to study its pathogenesis, epidemiology, and treatment. Most of the reported literature about MMD is from Japan. The annual report of the Committee has accumulated many clinical as well as research data, and studies are ongoing. The incidence of reports on MMD is increasing in Western countries. It is important to understand the current status of MMD management with its unsolved problems. No standardized surgical method of choice has yet been established. One reason for this is the absence of standardized evaluation measures and protocols. Here, current evaluation measures for pre- and post-operative outcomes are reviewed, and novel therapeutic research trials are presented as potential future perspectives on this disease.


Moyamoya Disease Superficial Temporal Artery Oxygen Extraction Fraction Blood Volume Flow Karnofsky Performance Scale Score 
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.
    Takeuchi K, Shimizu K (1957) Hypogenesis of bilateral internal carotid artery. No To Shinkei 9:37–43Google Scholar
  2. 2.
    Matsushima T, Fujiwara S, Nagata S et al (1989) Surgical treatment for pediatric patients with moyamoya disease by indirect revascularization procedures (EDAS, EMS, EMAS). Acta Neurochir 98:135–140PubMedCrossRefGoogle Scholar
  3. 3.
    Matsushima T, Inoue T, Suzuki SO (1992) Surgical treatment of moyamoya disease in pediatric patients-comparison the results of indirect and direct revascularization procedures. Neurosurgery 31:401–405PubMedCrossRefGoogle Scholar
  4. 4.
    Kim SK, Wang KC, Kim IO et al (2002) Combined encephaloduroarteriosynangiosis and bifrontal encephalogaleo(periosteal)synangiosis in pediatric moyamoya disease. Neurosurgery 50:88–96PubMedGoogle Scholar
  5. 5.
    Czabanka M, Vajkoczy P, Schmiedek P (2009) Age-dependent revascularization patterns in the treatment of moyamoya disease in European patient population. Neurosurg Focus 26(4):E9CrossRefGoogle Scholar
  6. 6.
    Matsushima Y, Aoyagi M, Niimi Y et al (1990) Symptoms and their pattern of progression in childhood moyamoya disease. Brain Dev 12(6):784–789PubMedCrossRefGoogle Scholar
  7. 7.
    Horn P, Vajkoczy P, Schmiedek P et al (2004) Evaluation of extracranial-intracranial arterial bypass function with magnetic resonance angiography. Neuroradiology 46:723–729PubMedCrossRefGoogle Scholar
  8. 8.
    Houkin K, Nakayama N, Kuroda S et al (2005) Novel magnetic resonance angiography stage grading for moyamoya disease. Cerebrovasc Dis 20:347–354PubMedCrossRefGoogle Scholar
  9. 9.
    Perren F, Meairs S, Schmiedek P et al (2005) Power Doppler evaluation of revascularization in childhood moyamoya. Neurology 64:558–560PubMedCrossRefGoogle Scholar
  10. 10.
    Sato H, Sato N, Tamaki N et al (1990) Chronic low-perfusion state in children with moyamoya disease following revascularization. Childs Nerv Syst 6(3):166–171PubMedCrossRefGoogle Scholar
  11. 11.
    Ikezaki K, Matsushima T, Kuwabara Y et al (1994) Cerebral circulation and oxygen metabolism in childhood moyamoya disease: a perioperative positron emission tomography study. J Neurosurg 81:843–850PubMedCrossRefGoogle Scholar
  12. 12.
    Kuroda S, Houkin K (2008) Moyamoya disease: current concepts and future prospective. Lancet Neurol 7:1056–1066PubMedCrossRefGoogle Scholar
  13. 13.
    Guzman R, Lee M, Achrol A et al (2009) Clinical outcome after 450 revascularization procedures for moymoy disease. J Neurosurg. doi: 10.3171/2009.4.JNS081649Google Scholar
  14. 14.
    Veeravagu A, Guzman R, Path CG et al (2008) Moyamoya disease in pediatric patients: outcomes of neurosurgical interventions. Neurosurg Focus 24(2):1–9CrossRefGoogle Scholar
  15. 15.
    Yamashita T, Deguchi K, Nagotani S et al (2009) Gene and stem cell therapy in ischemic stroke. Cell Transplant Apr 29 Pii:CT-2061 [Epub ahead of print]Google Scholar
  16. 16.
    Toyama K, Honmou O, Harada K et al (2009) Therapeutic benefits of angiogenetic gene-modified human mesenchymal stem cells after cerebral ischemia. Exp Neurol 216(1):47–55PubMedCrossRefGoogle Scholar
  17. 17.
    Zhu W, Fan Y, Hao Q et al (2009) Postischemic IGF-1 gene transfer promotes neurovascular regeneration after experimental stroke. J Cereb Blood Flow Metab 29(9):1528–1537PubMedCrossRefGoogle Scholar
  18. 18.
    Chen C, Hu Q, Yan J et al (2009) Early inhibition of HIF-1alpha with small interfering RNA reduce ischemic-reperfused brain injury in rats. Neurobiol Dis 33(3):509–517PubMedCrossRefGoogle Scholar
  19. 19.
    Lee HJ, Kim MK, Kim HJ et al (2009) Human neural stem cells genetically modified to overexpress Akt1 provide neuroprotection and functional improvement in mouse stroke model. PLoS One 4(5):e5586PubMedCrossRefGoogle Scholar
  20. 20.
    Perren F, Horn P, Vajkoczy P et al (2005) Power Doppler imaging in detection of surgically induced indirect neoangiogenesis in adult moyamoya disease. J Neurosurg 103:869–872PubMedCrossRefGoogle Scholar

Copyright information

© Springer 2010

Authors and Affiliations

  1. 1.Division of Pediatric Neurosurgery, Pediatric Clinical Neuroscience CenterSeoul National University Children's Hospital, Seoul National University College of MedicineJongno-guRepublic of Korea

Personalised recommendations