Skip to main content
SpringerLink
Log in

Moyamoya Disease

  • Chapter
  • First Online:
Emergency Approaches to Neurosurgical Conditions

  • 1151 Accesses

Abstract

First described in 1957 by Drs. Takeuchi and Shimizu as “hypoplasia” of the bilateral internal carotid (ICA) arteries, moyamoya was once thought to be a rare disorder limited to those of Asian heritage (Takeuchi et al. Brain Nerve 9:37–43, 1957). It is now recognized to affect all ethnicities, usually at younger ages, and with high risks of stroke if left untreated. Large series of surgical outcomes over several years have shown that surgical treatment is safe and effective in preventing stroke in moyamoya patients with very few lifestyle limitations (Abla et al. Neurosurgery 73(3):430–439, 2013; Guzman et al. J Neurosurg 111(5):927–935, 2009; Kim et al. Ann Neurol 68(1):92–101, 2010; Scott et al. J Neurosurg 100(2 Suppl Pediatrics):142–149, 2004).

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Abla AA, et al. Surgical outcomes for moyamoya angiopathy at barrow neurological institute with comparison of adult indirect encephaloduroarteriosynangiosis bypass, adult direct superficial temporal artery-to-middle cerebral artery bypass, and pediatric bypass: 154 revascularization surgeries in 140 affected hemispheres. Neurosurgery. 2013;73(3):430–9. doi:10.1227/NEU.0000000000000017.

    Article  PubMed  Google Scholar 

  2. Achrol AS, et al. Pathophysiology and genetic factors in moyamoya disease. Neurosurg Focus. 2009;26(4):E4. doi:10.3171/2009.1.FOCUS08302.

    Article  PubMed  Google Scholar 

  3. Achrol AS, et al. In reply. The genetics of moyamoya disease: recent insights into the pathogenesis of the disease. Neurosurgery. 2013;72(2):E321–322. doi:10.1227/NEU.0b013e31827bc1c1.

    Article  PubMed  Google Scholar 

  4. Calviere L, et al. Executive dysfunction in adults with moyamoya disease is associated with increased diffusion in frontal white matter. J Neurol Neurosurg Psychiatry. 2012;83(6):591–3. doi:10.1136/jnnp-2011-301388.

    Article  PubMed  Google Scholar 

  5. Chiu D, et al. Clinical features of moyamoya disease in the United States. Stroke. 1998;29(7):1347–51.

    Article  CAS  PubMed  Google Scholar 

  6. 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:S11–18.

    Article  PubMed  Google Scholar 

  7. Dorfman LJ, et al. Moyamoya disease can masquerade as multiple sclerosis. Neurologist. 2012;18(6):398–403. doi:10.1097/NRL.0b013e31826a99a1.

    Article  PubMed  Google Scholar 

  8. Guzman R, et al. Clinical outcome after 450 revascularization procedures for moyamoya disease. Clinical article. J Neurosurg. 2009;111(5):927–35. doi:10.3171/2009.4.JNS081649.

    Article  PubMed  Google Scholar 

  9. Guzman R, et al. Direct bypass techniques for the treatment of pediatric moyamoya disease. Neurosurg Clin N Am. 2010;21(3):565–73. doi:10.1016/j.nec.2010.03.013.

    Article  PubMed  Google Scholar 

  10. Hallemeier CL, et al. Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke. 2006;37(6):1490–6. doi:10.1161/01.STR.0000221787.70503.ca.

    Article  PubMed  Google Scholar 

  11. 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. doi:10.1038/jhg.2010.132. jhg2010132 [pii].

    Article  CAS  PubMed  Google Scholar 

  12. Karzmark P, et al. Neurocognitive impairment in adults with moyamoya disease without stroke. Neurosurgery. 2012;70(3):634–8. doi:10.1227/NEU.0b013e3182320d1a.

    Article  PubMed  Google Scholar 

  13. Karzmark P, et al. Effect of moyamoya disease on neuropsychological functioning in adults. Neurosurgery. 2008;62(5):1048–51. doi:10.1227/01.neu.0000325866.29634.4c; discussion 1051–1042.

    Article  PubMed  Google Scholar 

  14. Kelly ME, et al. Progression of unilateral moyamoya disease: a clinical series. Cerebrovasc Dis. 2006;22(2–3):109–15. doi:10.1159/000093238.

    Article  PubMed  Google Scholar 

  15. Khan N, et al. Sex differences in clinical presentation and treatment outcomes in Moyamoya disease. Neurosurgery. 2012;71(3):587–93. doi:10.1227/NEU.0b013e3182600b3c; discussion 593.

    Article  PubMed  Google Scholar 

  16. Khan N, et al. Failure of primary percutaneous angioplasty and stenting in the prevention of ischemia in Moyamoya angiopathy. Cerebrovasc Dis. 2011;31(2):147–53. doi:10.1159/000320253.

    Article  PubMed  Google Scholar 

  17. Kim SK, et al. Pediatric moyamoya disease: an analysis of 410 consecutive cases. Ann Neurol. 2010;68(1):92–101. doi:10.1002/ana.21981.

    Article  PubMed  Google Scholar 

  18. 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. doi:10.1161/STROKEAHA.106.478297. doi: STROKEAHA.106.478297 [pii].

    Article  PubMed  Google Scholar 

  19. Kuroda S, et al. Incidence and clinical features of disease progression in adult moyamoya disease. Stroke. 2005;36(10):2148–53. doi:10.1161/01.STR.0000182256.32489.99.

    Article  PubMed  Google Scholar 

  20. Kurokawa T, et al. Prognosis of occlusive disease of the circle of Willis (moyamoya disease) in children. Pediatr Neurol. 1985;1(5):274–7. 0887-8994(85)90027-X [pii].

    Article  CAS  PubMed  Google Scholar 

  21. Miskinyte S, et al. Loss of BRCC3 deubiquitinating enzyme leads to abnormal angiogenesis and is associated with syndromic moyamoya. Am J Hum Genet. 2011;88(6):718–28. doi:10.1016/j.ajhg.2011.04.017. S0002-9297(11)00158-3 [pii].

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  22. Navarro R, et al. Less-invasive pedicled omental-cranial transposition in pediatric patients with moyamoya disease and failed prior revascularization. Neurosurgery. 2013. doi:10.1227/NEU.0000000000000119.

    Google Scholar 

  23. Pandey P, et al. Patients with moyamoya disease presenting with movement disorder. J Neurosurg Pediatr. 2010;6(6):559–66. doi:10.3171/2010.9.PEDS10192.

    Article  PubMed  Google Scholar 

  24. Scott RM, et al. Moyamoya disease and moyamoya syndrome. N Engl J Med. 2009;360(12):1226–37. doi:10.1056/NEJMra0804622.

    Article  CAS  PubMed  Google Scholar 

  25. Scott RM, et al. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg. 2004;100(2 Suppl Pediatrics):142–9. doi:10.3171/ped.2004.100.2.0142.

    PubMed  Google Scholar 

  26. Smith ER, et al. Moyamoya: epidemiology, presentation, and diagnosis. Neurosurg Clin N Am. 2010;21(3):543–51. doi:10.1016/j.nec.2010.03.007.

    Article  PubMed  Google Scholar 

  27. Starke RM, et al. Moyamoya disorder in the United States. Neurosurgery. 2012;71(1):93–9. doi:10.1227/NEU.0b013e318253ab8e.

    Article  PubMed  Google Scholar 

  28. Starke RM, et al. Clinical features, surgical treatment, and long-term outcome in adult patients with moyamoya disease. Clinical article. J Neurosurg. 2009;111(5):936–42. doi:10.3171/2009.3.JNS08837.

    Article  PubMed Central  PubMed  Google Scholar 

  29. Suzuki J, et al. 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 

  30. Takeuchi K, et al. Hypoplasia of the bilateral internal carotid arteries. Brain Nerve. 1957;9:37–43.

    Google Scholar 

  31. Zach V, et al. Headache associated with moyamoya disease: a case story and literature review. J Headache Pain. 2010;11(1):79–82. doi:10.1007/s10194-009-0181-8.

    Article  PubMed Central  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA, 94305-5327, USA

    Teresa E. Bell-Stephens RN, BSN, CNRN & Gary K. Steinberg MD, PhD

Authors
  1. Teresa E. Bell-Stephens RN, BSN, CNRN
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Gary K. Steinberg MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Gary K. Steinberg MD, PhD .

Editor information

Editors and Affiliations

  1. Neurosurgery/Radiology, Brigham and Women's Medical Hospita Harvard Medical School, Boston, Massachusetts, USA

    Abhishek Agrawal

  2. Department of Neurosurgery, Methodist Neurological Institute, Houston, Texas, USA

    Gavin Britz

Additional information

Funding Sources

Josef Huber Family Moyamoya Fund, Stanley and Alexis Shin, Reddy Lee Moyamoya Fund, William Randolph Hearst Foundation, Bernard and Ronni Lacroute, Russell and Elizabeth Siegelman.

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Bell-Stephens, T.E., Steinberg, G.K. (2015). Moyamoya Disease. In: Agrawal, A., Britz, G. (eds) Emergency Approaches to Neurosurgical Conditions. Springer, Cham. https://doi.org/10.1007/978-3-319-10693-9_10

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-10693-9_10

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-10692-2

  • Online ISBN: 978-3-319-10693-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation