Abstract
Moyamoya disease (MMD) was first described in the Japanese population, and remains significantly more common in Asia. The etiology of the disease remains unknown, but it is a chronic cerebrovascular disorder that manifests as progressive stenosis and occlusion of the large intracranial arteries, and formation of abnormal collateral vascular supply as a compensatory mechanism. The disease has since been recognized as a clinical entity in non-Asian populations, but its true incidence in these populations remains unclear. MMD in the USA is of growing interest in recent years, and multiple authors have contributed series of long-term outcomes after treatment for MMD, which are now available.
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Abbreviations
- CBF:
-
cerebral blood flow
- CT:
-
computed tomography
- DSA:
-
digital subtraction angiography
- DWI:
-
diffusion weighted imaging
- ECIC:
-
extracranial-intracranial
- EDAS:
-
Encephaloduroarteriosynangiosis
- ICA:
-
internal carotid artery
- ICH:
-
intracerebral hemorrhage
- IPH:
-
intraparenchymal hemorrhage
- IVH:
-
intraventricular hemorrhage
- MAP:
-
mean arterial pressure
- MMD:
-
moyamoya disease
- MRI:
-
magnetic resonance imaging
- mRS:
-
modified Rankin Score
- OR:
-
odds ratio
- PET:
-
positron emission tomography
- SAH:
-
subarachnoid hemorrhage
- SPECT:
-
single photon emission computed tomography
- STA:
-
superficial temporal artery
- TIA:
-
transient ischemic attack
References
Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969;20(3):288–99.
Achrol AS, Guzman R, Lee M, Steinberg GK. Pathophysiology and genetic factors in moyamoya disease. Neurosurg Focus. 2009;26(4):E4.
Kuroda S, Houkin K. Moyamoya disease: current concepts and future perspectives. Lancet Neurol. 2008;7(11):1056–66.
Takekawa Y, Umezawa T, Ueno Y, Sawada T, Kobayashi M. Pathological and immunohistochemical findings of an autopsy case of adult moyamoya disease. Neuropathology. 2004;24(3):236–42.
Weinberg DG, Arnaout OM, Rahme RJ, Aoun SG, Batjer HH, Bendok BR. Moyamoya disease: a review of histopathology, biochemistry, and genetics. Neurosurg Focus. 2011;30(6):E20.
Uchino K, Johnston SC, Becker KJ, Tirschwell DL. Moyamoya disease in Washington state and California. Neurology. 2005;65(6):956–8.
Starke RM, Crowley RW, Maltenfort M, Jabbour PM, Gonzalez LF, Tjoumakaris SI, et al. Moyamoya disorder in the United States. Neurosurgery. 2012;71(1):93–9.
Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry. 2008;79(8):900–4.
Kuriyama S, Kusaka Y, Fujimura M, Wakai K, Tamakoshi A, Hashimoto S, et al. Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey. Stroke. 2008;39(1):42–7.
Graham JF. Matoba a. a survey of moyamoya disease in Hawaii. Clin Neurol Neurosurg. 1997;99(Suppl 2):S31–5.
Guzman R, Lee M, Achrol A, Bell-Stephens T, Kelly M, Do HM, et al. Clinical outcome after 450 revascularization procedures for moyamoya disease. Clinical article. J Neurosurg. 2009;111(5):927–35.
Feghali J, Xu R, Yang W, Liew J, Tamargo RJ, Marsh EB, et al. Racial phenotypes in moyamoya disease: a comparative analysis of clinical presentation and natural history in a single multiethnic cohort of 250 hemispheres. J Neurosurg. 2019:1–7.
Chiu D, Shedden P, Bratina P, Grotta JC. Clinical features of moyamoya disease in the United States. Stroke. 1998;29(7):1347–51.
Hallemeier CL, Rich KM, Grubb RL Jr, Chicoine MR, Moran CJ, Cross DT 3rd, et al. Clinical features and outcome in north American adults with moyamoya phenomenon. Stroke. 2006;37(6):1490–6.
Winkler EA, Yue JK, Deng H, Raygor KP, Phelps RRL, Rutledge C, et al. National trends in cerebral bypass surgery in the United States, 2002–2014. Neurosurg Focus. 2019;46(2):E4.
Abla AA, Gandhoke G, Clark JC, Oppenlander ME, Velat GJ, Zabramski JM, 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.
Titsworth WL, Scott RM, Smith ER. National Analysis of 2454 pediatric Moyamoya admissions and the effect of hospital volume on outcomes. Stroke. 2016;47(5):1303–11.
Bao XY, Duan L, Yang WZ, Li DS, Sun WJ, Zhang ZS, et al. Clinical features, surgical treatment, and long-term outcome in pediatric patients with moyamoya disease in China. Cerebrovasc Dis. 2015;39(2):75–81.
Mineharu Y, Takenaka K, Yamakawa H, Inoue K, Ikeda H, Kikuta KI, et al. Inheritance pattern of familial moyamoya disease: autosomal dominant mode and genomic imprinting. J Neurol Neurosurg Psychiatry. 2006;77(9):1025–9.
Gaillard J, Klein J, Duran D, Storey A, Scott RM, Kahle K, et al. Incidence, clinical features, and treatment of familial moyamoya in pediatric patients: a single-institution series. J Neurosurg Pediatr. 2017;19(5):553–9.
Ikezaki K, Han DH, Kawano T, Inamura T, Fukui M. Epidemiological survey of moyamoya disease in Korea. Clin Neurol Neurosurg. 1997;99(Suppl 2):S6–10.
Guzman R, Khan N, Steinberg GK. Moyamoya disease in North America. In: Moyamoya disease update [Internet]. Tokyo: Springer; 2010.
Scott RM, Smith JL, Robertson RL, Madsen JR, Soriano SG, Rockoff MA. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg. 2004;100(2 Suppl Pediatrics):142–9.
Iwama T, Yoshimura S. Present status of Moyamoya disease in Japan. Acta Neurochir Suppl. 2008;103:115–8.
Seol HJ, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho BK. Headache in pediatric moyamoya disease: review of 204 consecutive cases. J Neurosurg. 2005;103(5 Suppl):439–42.
Olesen J, Friberg L, Olsen TS, Andersen AR, Lassen NA, Hansen PE, et al. Ischaemia-induced (symptomatic) migraine attacks may be more frequent than migraine-induced ischaemic insults. Brain. 1993;116(Pt 1):187–202.
Park-Matsumoto YC, Tazawa T, Shimizu J. Migraine with aura-like headache associated with moyamoya disease. Acta Neurol Scand. 1999;100(2):119–21.
Kuroda S, Hashimoto N, Yoshimoto T, Iwasaki Y. Research committee on Moyamoya disease in J. radiological findings, clinical course, and outcome in asymptomatic moyamoya disease: results of multicenter survey in Japan. Stroke. 2007;38(5):1430–5.
Kuroda S, Ishikawa T, Houkin K, Nanba R, Hokari M, Iwasaki Y. Incidence and clinical features of disease progression in adult moyamoya disease. Stroke. 2005;36(10):2148–53.
Church EW, Bell-Stephens TE, Bigder MG, Gummidipundi S, Han SS, Steinberg GK. Clinical course of unilateral Moyamoya disease. Neurosurgery. 2020; https://doi.org/10.1093/neuros/nyaa293.
Veeravagu A, Guzman R, Patil CG, Hou LC, Lee M, Steinberg GK. Moyamoya disease in pediatric patients: outcomes of neurosurgical interventions. Neurosurg Focus. 2008;24(2):E16.
Agarwalla PK, Stapleton CJ, Phillips MT, Walcott BP, Venteicher AS, Ogilvy CS. Surgical outcomes following encephaloduroarteriosynangiosis in north American adults with moyamoya. J Neurosurg. 2014;121(6):1394–400.
Gonzalez NR, Dusick JR, Connolly M, Bounni F, Martin NA, Van de Wiele B, et al. Encephaloduroarteriosynangiosis for adult intracranial arterial steno-occlusive disease: long-term single-center experience with 107 operations. J Neurosurg. 2015;123(3):654–61.
Riordan CP, Storey A, Cote DJ, Smith ER, Scott RM. Results of more than 20 years of follow-up in pediatric patients with moyamoya disease undergoing pial synangiosis. J Neurosurg Pediatr. 2019:1–7.
Yu J, Shi L, Guo Y, Xu B, Xu K. Progress on complications of direct bypass for Moyamoya disease. Int J Med Sci. 2016;13(8):578–87.
Nielsen TH, Abhinav K, Sussman ES, Han SS, Weng Y, Bell-Stephens T, et al. Direct versus indirect bypass procedure for the treatment of ischemic moyamoya disease: results of an individualized selection strategy. J Neurosurg. 2020:1–12.
Teo MK, Madhugiri VS, Steinberg GK. Editorial: direct versus indirect bypass for moyamoya disease: ongoing controversy. J Neurosurg. 2017;126(5):1520–2.
Teo M, Johnson J, Steinberg GK. Strategies for and outcome of repeat revascularization surgery for Moyamoya disease: an American institutional series. Neurosurgery. 2017;81(5):852–9.
Mesiwala AH, Sviri G, Fatemi N, Britz GW, Newell DW. Long-term outcome of superficial temporal artery-middle cerebral artery bypass for patients with moyamoya disease in the US. Neurosurg Focus. 2008;24(2):E15.
Starke RM, Komotar RJ, Hickman ZL, Paz YE, Pugliese AG, Otten ML, 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.
Feghali J, Xu R, Yang W, Liew J, Tamargo RJ, Marsh EB, et al. Differing surgical outcomes in a multiethnic cohort suggest racial phenotypes in Moyamoya disease. World Neurosurg. 2019;128:e865–e72.
Sakamoto H, Kitano S, Yasui T, Komiyama M, Nishikawa M, Iwai Y, et al. Direct extracranial-intracranial bypass for children with moyamoya disease. Clin Neurol Neurosurg. 1997;99(Suppl 2):S128–33.
Fujimura M, Kaneta T, Tominaga T. Efficacy of superficial temporal artery-middle cerebral artery anastomosis with routine postoperative cerebral blood flow measurement during the acute stage in childhood moyamoya disease. Childs Nerv Syst. 2008;24(7):827–32.
Lopez JR. Neurophysiologic intraoperative monitoring of pediatric cerebrovascular surgery. J Clin Neurophysiol. 2009;26(2):85–94.
Choi R, Andres RH, Steinberg GK, Guzman R. Intraoperative hypothermia during vascular neurosurgical procedures. Neurosurg Focus. 2009;26(5):E24.
Iwama T, Hashimoto N, Yonekawa Y. The relevance of hemodynamic factors to perioperative ischemic complications in childhood moyamoya disease. Neurosurgery. 1996;38(6):1120–5. discussion 5–6
Teo M, Furtado S, Kaneko OF, Azad TD, Madhugiri V, Do HM, et al. Validation and application for the Berlin grading system of Moyamoya disease in adult patients. Neurosurgery. 2020;86(2):203–12.
Jea A, Smith ER, Robertson R, Scott RM. Moyamoya syndrome associated with down syndrome: outcome after surgical revascularization. Pediatrics. 2005;116(5):e694–701.
Yilmaz EY, Pritz MB, Bruno A, Lopez-Yunez A, Moyamoya BJ. Indiana university medical center experience. Arch Neurol. 2001;58(8):1274–8.
Lin N, Aronson JP, Manjila S, Smith ER, Scott RM. Treatment of Moyamoya disease in the adult population with pial synangiosis. J Neurosurg. 2014;120(3):612–7.
Abhinav K, Furtado SV, Nielsen TH, Iyer A, Gooderham PA, Teo M, et al. Functional outcomes after revascularization procedures in patients with hemorrhagic Moyamoya disease. Neurosurgery. 2020;86(2):257–65.
Ikezaki K, Inamura T, Kawano T, Fukui M. Clinical features of probable moyamoya disease in Japan. Clin Neurol Neurosurg. 1997;99(Suppl 2):S173–7.
Zeifert PD, Karzmark P, Bell-Stephens TE, Steinberg GK, Dorfman LJ. Neurocognitive performance after cerebral revascularization in adult Moyamoya disease. Stroke. 2017;48(6):1514–7.
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Ahmed, S.U., Steinberg, G.K. (2021). Long-Term Outcomes in the USA. In: Kuroda, S. (eds) Moyamoya Disease: Current Knowledge and Future Perspectives. Springer, Singapore. https://doi.org/10.1007/978-981-33-6404-2_21
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DOI: https://doi.org/10.1007/978-981-33-6404-2_21
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