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Moyamoya Disease

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Introduction to Vascular Neurosurgery

Abstract

A rare but important source of neurological morbidity, moyamoya disease, is a chronic, progressive, steno-occlusive disorder at the distal carotid and proximal middle and anterior cerebral arteries. Intracranial ischemia and hemorrhage are the two main clinical manifestations associated with this disease. The exact underlying mechanism of moyamoya disease remains unknown. Characteristic radiographic findings confirm the diagnosis and facilitate clinical staging. Optimal patient outcomes require early recognition of the disease and prompt institution of therapy. Evidence suggests that surgical revascularization is the only effective treatment for reducing the risk of ischemic and hemorrhagic stroke in moyamoya patients. Direct surgical revascularization by means of an extracranial-to-intracranial bypass and/or indirect revascularization through encephaloduroarteriosynangiosis (EDAS), encephalomyoarteriosynangiosis (EMS), and/or cncephaloduroarteriomyosynangiosis (EDAMS) are the main surgical modalities.

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References

  1. Suzuki J, Takaku A. Cerebrovascular "moyamoya" disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969;20(3):288–99.

    Article  CAS  PubMed  Google Scholar 

  2. Ahn IM, et al. Incidence, prevalence, and survival of moyamoya disease in Korea: a nationwide, population-based study. Stroke. 2014;45(4):1090–5.

    Article  PubMed  Google Scholar 

  3. Wakai K, et al. Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg. 1997;99(Suppl 2):S1–5.

    Article  PubMed  Google Scholar 

  4. Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry. 2008;79(8):900–4.

    Article  CAS  PubMed  Google Scholar 

  5. Kim SJ, et al. Neovascularization precedes occlusion in moyamoya disease: angiographic findings in 172 pediatric patients. Eur Neurol. 2014;72(5–6):299–305.

    Article  CAS  PubMed  Google Scholar 

  6. Bang OY, Fujimura M, Kim SK. The pathophysiology of Moyamoya disease: an update. J Stroke. 2016;18(1):12–20.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Kamada F, et al. A genome-wide association study identifies RNF213 as the first Moyamoya disease gene. J Hum Genet. 2011;56(1):34–40.

    Article  CAS  PubMed  Google Scholar 

  8. Miyatake S, et al. Homozygous c.14576G>A variant of RNF213 predicts early-onset and severe form of moyamoya disease. Neurology. 2012;78(11):803–10.

    Article  CAS  PubMed  Google Scholar 

  9. Wu Z, et al. Molecular analysis of RNF213 gene for moyamoya disease in the Chinese Han population. PLoS One. 2012;7(10):e48179.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Liu J, et al. Caveolin-1 expression enhances endothelial capillary tubule formation. J Biol Chem. 2002;277(12):10661–8.

    Article  CAS  PubMed  Google Scholar 

  11. Chang SH, et al. Vascular permeability and pathological angiogenesis in caveolin-1-null mice. Am J Pathol. 2009;175(4):1768–76.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Guo DC, et al. Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and Moyamoya disease, along with thoracic aortic disease. Am J Hum Genet. 2009;84(5):617–27.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Kang HS, et al. Smooth-muscle progenitor cells isolated from patients with moyamoya disease: novel experimental cell model. J Neurosurg. 2014;120(2):415–25.

    Article  CAS  PubMed  Google Scholar 

  14. Fukui M. 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. 1997;99(Suppl 2):S238–40.

    Article  PubMed  Google Scholar 

  15. Maeda M, Tsuchida C. "Ivy sign" on fluid-attenuated inversion-recovery images in childhood moyamoya disease. AJNR Am J Neuroradiol. 1999;20(10):1836–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  16. Yamada I, Suzuki S, Matsushima Y. Moyamoya disease: comparison of assessment with MR angiography and MR imaging versus conventional angiography. Radiology. 1995;196(1):211–8.

    Article  CAS  PubMed  Google Scholar 

  17. Kim JS. Moyamoya disease: epidemiology, clinical features, and diagnosis. J Stroke. 2016;18(1):2–11.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Lee S, et al. Moyamoya disease in children: results from the international pediatric stroke study. J Child Neurol. 2017;32(11):924–9.

    Article  PubMed  Google Scholar 

  19. Guzman R, et al. Clinical outcome after 450 revascularization procedures for moyamoya disease. Clinical article. J Neurosurg. 2009;111(5):927–35.

    Article  PubMed  Google Scholar 

  20. Suzuki J, Kodama N. Moyamoya disease--a review. Stroke. 1983;14(1):104–9.

    Article  CAS  PubMed  Google Scholar 

  21. Kim SK, et al. Pediatric moyamoya disease: an analysis of 410 consecutive cases. Ann Neurol. 2010;68(1):92–101.

    Article  PubMed  Google Scholar 

  22. Kim JM, Lee SH, Roh JK. Changing ischaemic lesion patterns in adult moyamoya disease. J Neurol Neurosurg Psychiatry. 2009;80(1):36–40.

    Article  PubMed  Google Scholar 

  23. Hishikawa T, et al. Assessment of the difference in posterior circulation involvement between pediatric and adult patients with moyamoya disease. J Neurosurg. 2013;119(4):961–5.

    Article  PubMed  Google Scholar 

  24. Cho HJ, et al. The different infarct patterns between adulthood-onset and childhood-onset moyamoya disease. J Neurol Neurosurg Psychiatry. 2011;82(1):38–40.

    Article  CAS  PubMed  Google Scholar 

  25. Nah HW, et al. Moyamoya disease-related versus primary intracerebral hemorrhage: [corrected] location and outcomes are different. Stroke. 2012;43(7):1947–50.

    Article  PubMed  Google Scholar 

  26. Hogan AM, et al. Intellectual decline in children with moyamoya and sickle cell anaemia. Dev Med Child Neurol. 2005;47(12):824–9.

    Article  CAS  PubMed  Google Scholar 

  27. Imaizumi C, et al. Serial intelligence test scores in pediatric moyamoya disease. Neuropediatrics. 1999;30(6):294–9.

    Article  CAS  PubMed  Google Scholar 

  28. Seol HJ, et al. Headache in pediatric moyamoya disease: review of 204 consecutive cases. J Neurosurg. 2005;103(5 Suppl):439–42.

    PubMed  Google Scholar 

  29. Kuroda S, Houkin K. Moyamoya disease: current concepts and future perspectives. Lancet Neurol. 2008;7(11):1056–66.

    Article  PubMed  Google Scholar 

  30. Choi JU, et al. Natural history of moyamoya disease: comparison of activity of daily living in surgery and non surgery groups. Clin Neurol Neurosurg. 1997;99(Suppl 2):S11–8.

    Article  PubMed  Google Scholar 

  31. Cho WS, et al. The natural clinical course of hemodynamically stable adult moyamoya disease. J Neurosurg. 2015;122(1):82–9.

    Article  PubMed  Google Scholar 

  32. Kang S, et al. Natural course of moyamoya disease in patients with prior hemorrhagic stroke. Stroke. 2019;50(5):1060–6.

    Article  PubMed  Google Scholar 

  33. Su IC, et al. Acute cerebral ischemia following intraventricular hemorrhage in moyamoya disease: early perfusion computed tomography findings. J Neurosurg. 2008;109(6):1049–51.

    Article  PubMed  Google Scholar 

  34. Kurokawa T, et al. Prognosis of occlusive disease of the circle of Willis (moyamoya disease) in children. Pediatr Neurol. 1985;1(5):274–7.

    Article  CAS  PubMed  Google Scholar 

  35. Ishii K, et al. Temporal profile of angiographical stages of moyamoya disease: when does moyamoya disease progress? Neurol Res. 2003;25(4):405–10.

    Article  PubMed  Google Scholar 

  36. Kelly ME, et al. Progression of unilateral moyamoya disease: a clinical series. Cerebrovasc Dis. 2006;22(2–3):109–15.

    Article  PubMed  Google Scholar 

  37. Lee M, et al. Quantitative hemodynamic studies in moyamoya disease: a review. Neurosurg Focus. 2009;26(4):E5.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Kuroda S, et al. Radiological findings, clinical course, and outcome in asymptomatic moyamoya disease: results of multicenter survey in Japan. Stroke. 2007;38(5):1430–5.

    Article  PubMed  Google Scholar 

  39. Neff KW, et al. 2D cine phase-contrast MRI for volume flow evaluation of the brain-supplying circulation in moyamoya disease. AJR Am J Roentgenol. 2006;187(1):W107–15.

    Article  PubMed  Google Scholar 

  40. Ogasawara K, Ogawa A, Yoshimoto T. Cerebrovascular reactivity to acetazolamide and outcome in patients with symptomatic internal carotid or middle cerebral artery occlusion: a xenon-133 single-photon emission computed tomography study. Stroke. 2002;33(7):1857–62.

    Article  PubMed  Google Scholar 

  41. Logothetis NK, Pfeuffer J. On the nature of the BOLD fMRI contrast mechanism. Magn Reson Imaging. 2004;22(10):1517–31.

    Article  PubMed  Google Scholar 

  42. Czabanka M, et al. Proposal for a new grading of Moyamoya disease in adult patients. Cerebrovasc Dis. 2011;32(1):41–50.

    Article  CAS  PubMed  Google Scholar 

  43. Horn P, et al. Hemodynamic reserve and high-intensity transient signals in moyamoya disease. Cerebrovasc Dis. 2005;19(3):141–6.

    Article  PubMed  Google Scholar 

  44. Chen J, et al. Microembolic signals predict cerebral ischaemic events in patients with moyamoya disease. Eur J Neurol. 2014;21(5):785–90.

    Article  CAS  PubMed  Google Scholar 

  45. Kraemer M, et al. What is the expert's option on antiplatelet therapy in moyamoya disease? Results of a worldwide Survey. Eur J Neurol. 2012;19(1):163–7.

    Article  CAS  PubMed  Google Scholar 

  46. Im SH, et al. Moyamoya disease associated with Graves disease: special considerations regarding clinical significance and management. J Neurosurg. 2005;102(6):1013–7.

    Article  PubMed  Google Scholar 

  47. Czartoski T, et al. Postinfectious vasculopathy with evolution to moyamoya syndrome. J Neurol Neurosurg Psychiatry. 2005;76(2):256–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Hallemeier CL, et al. Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke. 2006;37(6):1490–6.

    Article  PubMed  Google Scholar 

  49. Kraemer M, Heienbrok W, Berlit P. Moyamoya disease in Europeans. Stroke. 2008;39(12):3193–200.

    Article  PubMed  Google Scholar 

  50. Tu YK, et al. Combined encephalo-arterio-synangiosis and encephalo-myo-synangiosis in the treatment of moyamoya disease. Clin Neurol Neurosurg. 1997;99(Suppl 2):S118–22.

    Article  PubMed  Google Scholar 

  51. Kim T, et al. Stroke prevention by direct revascularization for patients with adult-onset moyamoya disease presenting with ischemia. J Neurosurg. 2016;124(6):1788–93.

    Article  PubMed  Google Scholar 

  52. Fujii K, et al. The efficacy of bypass surgery for the patients with hemorrhagic moyamoya disease. Clin Neurol Neurosurg. 1997;99(Suppl 2):S194–5.

    Article  PubMed  Google Scholar 

  53. Kawaguchi S, Okuno S, Sakaki T. Effect of direct arterial bypass on the prevention of future stroke in patients with the hemorrhagic variety of moyamoya disease. J Neurosurg. 2000;93(3):397–401.

    Article  CAS  PubMed  Google Scholar 

  54. Lee SB, et al. Long-term follow-up results in 142 adult patients with moyamoya disease according to management modality. Acta Neurochir. 2012;154(7):1179–87.

    Article  PubMed  Google Scholar 

  55. Qian C, et al. The efficacy of surgical treatment for the secondary prevention of stroke in symptomatic moyamoya disease: a meta-analysis. Medicine (Baltimore). 2015;94(49):e2218.

    Article  Google Scholar 

  56. Gross BA, Thomas AJ, Frerichs KU. Endovascular treatment of symptomatic moyamoya. Neurosurg Rev. 2014;37(4):579–83.

    Article  PubMed  Google Scholar 

  57. El-Hakam LM, et al. Angioplasty for acute stroke with pediatric moyamoya syndrome. J Child Neurol. 2010;25(10):1278–83.

    Article  PubMed  Google Scholar 

  58. LoPresti MA, et al. Endovascular stenting for rescue of a failed donor graft during superficial temporal artery to middle cerebral artery bypass surgery: case report. J Neurosurg Pediatr. 2019:1–5.

    Google Scholar 

  59. Goethe EA, et al. Endovascular "boost": external carotid angioplasty to augment flow before superficial temporal-middle cerebral artery bypass. World Neurosurg. 2019;130:474–8.

    Article  PubMed  Google Scholar 

  60. Kubota Y, et al. T-configuration stent placement for carotid bifurcation stenosis co-existing with ipsilateral intracranial stenosis: a case report and literature review. J Stroke Cerebrovasc Dis. 2020;30(2):105472.

    Article  PubMed  Google Scholar 

  61. Kim SH, et al. Endovascular treatment of ruptured aneurysms or pseudoaneurysms on the collateral vessels in patients with moyamoya disease. Neurosurgery. 2009;65(5):1000–4; discussion 1004

    Article  PubMed  Google Scholar 

  62. Kuriyama S, et al. Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey. Stroke. 2008;39(1):42–7.

    Article  PubMed  Google Scholar 

  63. Im SH, et al. Prevalence and epidemiological features of moyamoya disease in Korea. J Cerebrovasc Endovasc Neurosurg. 2012;14(2):75–8.

    Article  PubMed  Google Scholar 

  64. Chen PC, et al. Epidemiology of moyamoya disease in Taiwan: a nationwide population-based study. Stroke. 2014;45(5):1258–63.

    Article  PubMed  Google Scholar 

  65. Uchino K, et al. Moyamoya disease in Washington State and California. Neurology. 2005;65(6):956–8.

    Article  PubMed  Google Scholar 

  66. Wanebo JE, Khan N, Zabramski JM, Spetzler RF. Moyamoya disease: diagnosis and treatment [Internet]. Stuttgart: Thieme; 2014. Available from: https://doi.org/10.1055/b-002-91663

    Book  Google Scholar 

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Kvint, S., Burkhardt, JK. (2022). Moyamoya Disease. In: Mascitelli, J.R., Binning, M.J. (eds) Introduction to Vascular Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-030-88196-2_15

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  • DOI: https://doi.org/10.1007/978-3-030-88196-2_15

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