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Moyamoya Disease (Spontaneous Occlusion of the Circle of Willis)

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Abstract

Moyamoya is a disease affecting the cerebral arteries, which predisposes individuals to recurrent ischemic attack in association with progressive steno-occlusive change of the intracranial internal carotid arteries and their proximal branches. The disorder is typically characterized by a reduction in blood flow in the major vessels of the anterior circulation of the brain. This results in the development of a compensatory collateral vasculature around the stenosed vessels, near the apex of the internal carotid, on the cortical surface, leptomeninges, and branches of the external carotid artery and the base of the skull. In advanced cases, the posterior circulation is also involved, including the basilar and posterior cerebral arteries. While it was first described as a “hypoplasia of the bilateral internal carotid arteries,” the characteristic appearance of the network of abnormally dilated collateral vessels on conventional angiography was compared to “a puff of cigarette smoke” (Arch Neurol 20(3):288–899, 1969) or “moyamoya” in Japanese. The designation “spontaneous occlusion of the circle of Willis” was recently suggested as an alternative by the International Classification of Diseases (ICD). Moyamoya disease patients are predominantly found in Eastern Asian countries such as Japan and the Republic of Korea. Moyamoya disease patients show bimodal distribution, adult type and pediatric type. Symptom of adult moyamoya patients can be either ischemic or hemorrhagic; nevertheless, pediatric patients usually present with ischemia.

In ischemic moyamoya disease, the therapeutic effect of surgical revascularization (extracranial-intracranial bypass) has been well established. Bypass surgery can improve the impaired cerebral hemodynamic state and decrease the recurrent ischemic events. Management of the hemorrhagic moyamoya disease, on the other hand, presents a serious challenge. Despite the extremely high rate of rebleeding attacks, no therapeutic method has been established until recently. In 2013, the Japan Adult Moyamoya (JAM) Trial, a multicentered prospective randomized controlled trial, was completed. It has been revealed that bypass surgery significantly decreases the rate of rebleeding attacks and improve the patients’ prognosis during the following 5 years. This epoch-making study is expected to establish a guiding principle for the treatment of hemorrhagic moyamoya disease.

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References

  1. Suzuki J, Takaku A (1969) Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20(3):288–299, PubMed PMID: 5775283

    CAS  PubMed  Google Scholar 

  2. Miyamoto S, Yoshimoto T, Hashimoto N, Okada Y, Tsuji I, Tominaga T et al (2014) Effects of extracranial-intracranial bypass for patients with hemorrhagic moyamoya disease: results of the Japan Adult Moyamoya Trial. Stroke 45(5):1415–1421, PubMed PMID: 24668203. Eng

    PubMed  Google Scholar 

  3. Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis, Health Labour Sciences Research Grant for Research on Measures for Infractable Diseases, Hashimoto N et al (2012) Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion of the circle of Willis). Neurol Med Chir ;52(5):245–266. PubMed PMID: 22870528. Epub 2012/08/09. eng

    Google Scholar 

  4. Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R et al (1997) Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg 99(Suppl 2):S1–S5, PubMed PMID: 9409395. Epub 1997/12/31. eng

    PubMed  Google Scholar 

  5. Kleinloog R, Regli L, Rinkel GJ, Klijn CJ (2012) Regional differences in incidence and patient characteristics of moyamoya disease: a systematic review. J Neurol Neurosurg Psychiatry 83(5):531–536, PubMed PMID: 22378916. Epub 2012/03/02. eng

    PubMed  Google Scholar 

  6. Kuriyama S, Kusaka Y, Fujimura M, Wakai K, Tamakoshi A, Hashimoto S et al (2008) Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey. Stroke 39(1):42–47, PubMed PMID: 18048855. Epub 2007/12/01. eng

    PubMed  Google Scholar 

  7. Baba T, Houkin K, Kuroda S (2008) Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 79(8):900–904, PubMed PMID: 18077479. Epub 2007/12/14. eng

    CAS  PubMed  Google Scholar 

  8. Yamauchi T, Houkin K, Tada M, Abe H (1997) Familial occurrence of moyamoya disease. Clin Neurol Neurosurg 99(Suppl 2):S162–S167, PubMed PMID: 9409429. Epub 1997/12/31. eng

    PubMed  Google Scholar 

  9. Nanba R, Kuroda S, Tada M, Ishikawa T, Houkin K, Iwasaki Y (2006) Clinical features of familial moyamoya disease. Child’s Nerv Syst 22(3):258–262, PubMed PMID: 16195874. Epub 2005/10/01. eng

    Google Scholar 

  10. Houkin K, Yoshimoto T, Abe H, Nagashima K, Nagashima M, Takeda M et al (1998) Role of basic fibroblast growth factor in the pathogenesis of moyamoya disease. Neurosurg Focus 5(5):e2, PubMed PMID: 17112205. Epub 2006/11/23. eng

    CAS  PubMed  Google Scholar 

  11. Mineharu Y, Takenaka K, Yamakawa H, Inoue K, Ikeda H, Kikuta KI et al (2006) Inheritance pattern of familial moyamoya disease: autosomal dominant mode and genomic imprinting. J Neurol Neurosurg Psychiatry 77(9):1025–1029, PubMed PMID: 16788009. Pubmed Central PMCID: PMC2077755. Epub 2006/06/22. eng

    PubMed Central  CAS  PubMed  Google Scholar 

  12. Kamada F, Aoki Y, Narisawa A, Abe Y, Komatsuzaki S, Kikuchi A et al (2011) A genome-wide association study identifies RNF213 as the first moyamoya disease gene. J Hum Genet 56(1):34–40, PubMed PMID: 21048783. Epub 2010/11/05. eng

    CAS  PubMed  Google Scholar 

  13. Liu W, Morito D, Takashima S, Mineharu Y, Kobayashi H, Hitomi T et al (2011) Identification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development. PLoS One 6(7):e22542, PubMed PMID: 21799892. Pubmed Central PMCID: PMC3140517. Epub 2011/07/30. eng

    PubMed Central  CAS  PubMed  Google Scholar 

  14. Houkin K, Ito M, Sugiyama T, Shichinohe H, Nakayama N, Kazumata K et al (2012) Review of past research and current concepts on the etiology of moyamoya disease. Neurol Med Chir 52(5):267–277, PubMed PMID: 22688062. Epub 2012/06/13. eng

    Google Scholar 

  15. Kuroda S, Hashimoto N, Yoshimoto T, Iwasaki Y (2007) Radiological findings, clinical course, and outcome in asymptomatic moyamoya disease: results of multicenter survey in Japan. Stroke 38(5):1430–1435, PubMed PMID: 17395863. Epub 2007/03/31. eng

    PubMed  Google Scholar 

  16. Kuroda S (2010) Asymptomatic moyamoya disease. In: Cho B-K, Tominaga T (eds) Moyamoya disease update. Springer, Tokyo, pp 336–340

    Google Scholar 

  17. Kuroda S, Ishikawa T, Houkin K, Nanba R, Hokari M, Iwasaki Y (2005) Incidence and clinical features of disease progression in adult moyamoya disease. Stroke 36(10):2148–2153, PubMed PMID: 16179571. Epub 2005/09/24. eng

    PubMed  Google Scholar 

  18. Sawada T, Yamamoto A, Miki Y, Kikuta K, Okada T, Kanagaki M et al (2012) Diagnosis of moyamoya disease using 3-T MRI and MRA: value of cisternal moyamoya vessels. Neuroradiology 54(10):1089–1097, PubMed PMID: 22349623. Epub 2012/02/22

    PubMed  Google Scholar 

  19. 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 99(Suppl 2):S238–S240, PubMed PMID: 9409446. Epub 1997/12/31

    PubMed  Google Scholar 

  20. Houkin K, Abe H, Yoshimoto T, Takahashi A (1996) Is “unilateral” moyamoya disease different from moyamoya disease? J Neurosurg 85(5):772–776, PubMed PMID: 8893713. Epub 1996/11/01. eng

    CAS  PubMed  Google Scholar 

  21. Kawano T, Fukui M, Hashimoto N, Yonekawa Y (1994) Follow-up study of patients with “unilateral” moyamoya disease. Neurol Med Chir 34(11):744–747, PubMed PMID: 7533268. Epub 1994/11/01. eng

    CAS  Google Scholar 

  22. Kelly ME, Bell-Stephens TE, Marks MP, Do HM, Steinberg GK (2006) Progression of unilateral moyamoya disease: a clinical series. Cerebrovasc Dis (Basel, Switzerland) 22(2–3):109–115, PubMed PMID: 16685122. Epub 2006/05/11. eng

    Google Scholar 

  23. Cheong HI, Choi Y (2010) Systemic arterial involvement in moyamoya disease. In: Cho B-K, Tominaga T (eds) Moyamoya disease update. Springer, Tokyo, pp 126–131

    Google Scholar 

  24. Fukui M, Kono S, Sueishi K, Ikezaki K (2000) Moyamoya disease. Neuropathology 20(Suppl):S61–S64, PubMed PMID: 11037190. Epub 2000/10/19. eng

    PubMed  Google Scholar 

  25. Oka K, Yamashita M, Sadoshima S, Tanaka K (1981) Cerebral haemorrhage in moyamoya disease at autopsy. Virchows Arch A Pathol Anat Histol 392(3):247–261, PubMed PMID: 7269227. Epub 1981/01/01. eng

    CAS  PubMed  Google Scholar 

  26. Takagi Y, Kikuta K, Sadamasa N, Nozaki K, Hashimoto N (2006) Caspase-3-dependent apoptosis in middle cerebral arteries in patients with moyamoya disease. Neurosurgery 59(4):894–900, discussion -1. PubMed PMID: 17038954. Epub 2006/10/14. eng

    PubMed  Google Scholar 

  27. Yamashita M, Oka K, Tanaka K (1983) Histopathology of the brain vascular network in moyamoya disease. Stroke 14(1):50–58, PubMed PMID: 6823686. Epub 1983/01/01. eng

    CAS  PubMed  Google Scholar 

  28. Kuroda S, Houkin K (2008) Moyamoya disease: current concepts and future perspectives. Lancet Neurol 7(11):1056–1066, PubMed PMID: 18940695. Epub 2008/10/23. eng

    PubMed  Google Scholar 

  29. Karasawa J, Touho H, Ohnishi H, Miyamoto S, Kikuchi H (1993) Cerebral revascularization using omental transplantation for childhood moyamoya disease. J Neurosurg 79(2):192–196, PubMed PMID: 8331399. Epub 1993/08/01. eng

    CAS  PubMed  Google Scholar 

  30. Miyamoto S, Kikuchi H, Karasawa J, Nagata I, Ihara I, Yamagata S (1986) Study of the posterior circulation in moyamoya disease. Part 2: visual disturbances and surgical treatment. J Neurosurg 65(4):454–460, PubMed PMID: 3760953. Epub 1986/10/01. eng

    CAS  PubMed  Google Scholar 

  31. Kuroda S, Houkin K, Ishikawa T, Nakayama N, Ikeda J, Ishii N et al (2004) Determinants of intellectual outcome after surgical revascularization in pediatric moyamoya disease: a multivariate analysis. Child’s Nerv Syst 20(5):302–308, PubMed PMID: 15045517. Epub 2004/03/27. eng

    Google Scholar 

  32. Kodama N, Aoki Y, Hiraga H, Wada T, Suzuki J (1979) Electroencephalographic findings in children with moyamoya disease. Arch Neurol 36(1):16–19, PubMed PMID: 420596. Epub 1979/01/01. eng

    CAS  PubMed  Google Scholar 

  33. Kuroda S, Kamiyama H, Isobe M, Houkin K, Abe H, Mitsumori K (1995) Cerebral hemodynamics and “re-build-up” phenomenon on electroencephalogram in children with moyamoya disease. Child’s Nerv Syst 11(4):214–219, PubMed PMID: 7621482. Epub 1995/04/01. eng

    CAS  Google Scholar 

  34. Irikura K, Miyasaka Y, Kurata A, Tanaka R, Fujii K, Yada K et al (1996) A source of haemorrhage in adult patients with moyamoya disease: the significance of tributaries from the choroidal artery. Acta Neurochir 138(11):1282–1286, PubMed PMID: 8980730. Epub 1996/01/01. eng

    CAS  PubMed  Google Scholar 

  35. Kuroda S, Houkin K, Kamiyama H, Abe H (2001) Effects of surgical revascularization on peripheral artery aneurysms in moyamoya disease: report of three cases. Neurosurgery 49(2):463–467, PubMed PMID: 11504126. discussion 7–8. Epub 2001/08/16. eng

    CAS  PubMed  Google Scholar 

  36. Kawaguchi S, Sakaki T, Morimoto T, Kakizaki T, Kamada K (1996) Characteristics of intracranial aneurysms associated with moyamoya disease. A review of 111 cases. Acta Neurochir 138(11):1287–1294, PubMed PMID: 8980731. Epub 1996/01/01. eng

    CAS  PubMed  Google Scholar 

  37. Osanai T, Kuroda S, Nakayama N, Yamauchi T, Houkin K, Iwasaki Y (2008) Moyamoya disease presenting with subarachnoid hemorrhage localized over the frontal cortex: case report. Surg Neurol 69(2):197–200, PubMed PMID: 18261650. Epub 2008/02/12. eng

    PubMed  Google Scholar 

  38. Verret S, Steele JC (1971) Alternating hemiplegia in childhood: a report of eight patients with complicated migraine beginning in infancy. Pediatrics 47(4):675–680, PubMed PMID: 5089756. Epub 1971/04/01. eng

    CAS  PubMed  Google Scholar 

  39. Shirane R, Fujimura M (2010) Headache in moyamoya disease. In: Cho B-K, Tominaga T (eds) Moyamoya disease update. Springer, Tokyo, pp 110–113

    Google Scholar 

  40. Nogawa S, Suzuki N (2010) Involuntary movement. In: Cho B-K, Tominaga T (eds) Moyamoya disease update. Springer, Tokyo, pp 126–131

    Google Scholar 

  41. Gonzalez-Alegre P, Ammache Z, Davis PH, Rodnitzky RL (2003) Moyamoya-induced paroxysmal dyskinesia. Mov Disord 18(9):1051–1056, PubMed PMID: 14502675. Epub 2003/09/23. eng

    PubMed  Google Scholar 

  42. Nishimoto A, Takeuchi S (1968) Abnormal cerebrovascular network related to the internal carotid arteries. J Neurosurg 29(3):255–260, PubMed PMID: 5684407. Epub 1968/09/01. eng

    CAS  PubMed  Google Scholar 

  43. Houkin K, Iihoshi S, Mikami T (2010) Overview of image diagnosis of moyamoya disease. In: Cho B-K, Tominaga T (eds) Moyamoya disease update. Springer, Tokyo, pp 141–149

    Google Scholar 

  44. Sugino T, Mikami T, Ohtaki S, Hirano T, Iihoshi S, Houkin K et al (2013) Assessment of moyamoya disease using multidetector row computed tomography. J Stroke Cerebrovasc Dis 22(5):644–649, PubMed PMID: 22365710. Epub 2012/03/01. eng

    PubMed  Google Scholar 

  45. Houkin K, Nakayama N, Kuroda S, Nonaka T, Shonai T, Yoshimoto T (2005) Novel magnetic resonance angiography stage grading for moyamoya disease. Cerebrovasc Dis 20(5):347–354, PubMed PMID: 16131804. Epub 2005/09/01. eng

    PubMed  Google Scholar 

  46. Maeda M, Tsuchida C (1999) “Ivy sign” on fluid-attenuated inversion-recovery images in childhood moyamoya disease. AJNR Am J Neuroradiol 20(10):1836–1838, PubMed PMID: 10588105. Epub 1999/12/10

    CAS  PubMed  Google Scholar 

  47. Toyoda K, Ida M, Fukuda K (2001) Fluid-attenuated inversion recovery intraarterial signal: an early sign of hyperacute cerebral ischemia. AJNR Am J Neuroradiol 22(6):1021–1029, PubMed PMID: 11415892. Epub 2001/06/21. eng

    CAS  PubMed  Google Scholar 

  48. Takanashi J (2011) Moyamoya disease in children. Brain Dev 33(3):229–234, PubMed PMID: 20932697. Epub 2010/10/12. eng

    PubMed  Google Scholar 

  49. Kawashima M, Noguchi T, Takase Y, Ootsuka T, Kido N, Matsushima T (2009) Unilateral hemispheric proliferation of ivy sign on fluid-attenuated inversion recovery images in moyamoya disease correlates highly with ipsilateral hemispheric decrease of cerebrovascular reserve. AJNR Am J Neuroradiol 30(9):1709–1716, PubMed PMID: 19713323. Epub 2009/08/29. eng

    CAS  PubMed  Google Scholar 

  50. Kawashima M, Noguchi T, Takase Y, Nakahara Y, Matsushima T (2010) Decrease in leptomeningeal ivy sign on fluid-attenuated inversion recovery images after cerebral revascularization in patients with moyamoya disease. AJNR Am J Neuroradiol 31(9):1713–1718, PubMed PMID: 20466798. Epub 2010/05/15. eng

    CAS  PubMed  Google Scholar 

  51. Mori N, Mugikura S, Higano S, Kaneta T, Fujimura M, Umetsu A et al (2009) The leptomeningeal “ivy sign” on fluid-attenuated inversion recovery MR imaging in moyamoya disease: a sign of decreased cerebral vascular reserve? AJNR Am J Neuroradiol 30(5):930–935, PubMed PMID: 19246527. Epub 2009/02/28. eng

    CAS  PubMed  Google Scholar 

  52. Fushimi Y, Miki Y, Kikuta K, Okada T, Kanagaki M, Yamamoto A et al (2006) Comparison of 3.0- and 1.5-T three-dimensional time-of-flight MR angiography in moyamoya disease: preliminary experience. Radiology 239(1):232–237, PubMed PMID: 16467209. Epub 2006/02/10. eng

    PubMed  Google Scholar 

  53. Mori N, Miki Y, Kikuta K, Fushimi Y, Okada T, Urayama S et al (2008) Microbleeds in moyamoya disease: susceptibility-weighted imaging versus T2∗-weighted imaging at 3 Tesla. Invest Radiol 43(8):574–579, PubMed PMID: 18648257. Epub 2008/07/24. eng

    PubMed  Google Scholar 

  54. Kikuta K, Takagi Y, Nozaki K, Hanakawa T, Okada T, Mikuni N et al (2005) Asymptomatic microbleeds in moyamoya disease: T2∗-weighted gradient-echo magnetic resonance imaging study. J Neurosurg 102(3):470–475, PubMed PMID: 15796381. Epub 2005/03/31

    PubMed  Google Scholar 

  55. Horie N, Morikawa M, Nozaki A, Hayashi K, Suyama K, Nagata I (2011) “Brush Sign” on susceptibility-weighted MR imaging indicates the severity of moyamoya disease. AJNR Am J Neuroradiol 32(9):1697–1702, PubMed PMID: 21799039. Epub 2011/07/30. eng

    CAS  PubMed  Google Scholar 

  56. Hayashi K, Nagata I (2010) Progression of moyamoya disease. In: Cho B-K, Tominaga T (eds) Moyamoya disease update. Springer, Tokyo, pp 118–125

    Google Scholar 

  57. Suzuki J, Kodama N (1983) Moyamoya disease – a review. Stroke 14(1):104–109, PubMed PMID: 6823678. Epub 1983/01/01. eng

    CAS  PubMed  Google Scholar 

  58. Kodama N, Suzuki J (1978) Moyamoya disease associated with aneurysm. J Neurosurg 48(4):565–569, PubMed PMID: 632880. Epub 1978/04/01. eng

    CAS  PubMed  Google Scholar 

  59. Currie S, Raghavan A, Batty R, Connolly DJ, Griffiths PD (2011) Childhood moyamoya disease and moyamoya syndrome: a pictorial review. Pediatr Neurol 44(6):401–413, PubMed PMID: 21555050. Epub 2011/05/11

    PubMed  Google Scholar 

  60. Maki Y, Enomoto T (1988) Moyamoya disease. Child’s Nerv Syst 4(4):204–212, PubMed PMID: 3048657. Epub 1988/08/01

    CAS  Google Scholar 

  61. Yamada I, Suzuki S, Matsushima Y (1995) Moyamoya disease: comparison of assessment with MR angiography and MR imaging versus conventional angiography. Radiology 196(1):211–218, PubMed PMID: 7784569. Epub 1995/07/01. eng

    CAS  PubMed  Google Scholar 

  62. Tsuchiya K, Makita K, Furui S (1994) Moyamoya disease: diagnosis with three-dimensional CT angiography. Neuroradiology 36(6):432–434, PubMed PMID: 7991085. Epub 1994/08/01

    CAS  PubMed  Google Scholar 

  63. Kikuchi M, Asato M, Sugahara S, Nakajima K, Sato M, Nagao K et al (1996) Evaluation of surgically formed collateral circulation in moyamoya disease with 3D-CT angiography: comparison with MR angiography and X-ray angiography. Neuropediatrics 27(1):45–49, PubMed PMID: 8677026. Epub 1996/02/01

    CAS  PubMed  Google Scholar 

  64. Ogasawara K, Ogawa A, Yoshimoto T (2002) 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 33(7):1857–1862, PubMed PMID: 12105366. Epub 2002/07/10

    PubMed  Google Scholar 

  65. Nariai T, Matsushima Y, Imae S, Tanaka Y, Ishii K, Senda M et al (2005) Severe haemodynamic stress in selected subtypes of patients with moyamoya disease: a positron emission tomography study. J Neurol Neurosurg Psychiatry 76(5):663–669, PubMed PMID: 15834024. Pubmed Central PMCID: 1739646. Epub 2005/04/19. eng

    PubMed Central  CAS  PubMed  Google Scholar 

  66. Czabanka M, Pena-Tapia P, Schubert GA, Heppner FL, Martus P, Horn P et al (2011) Proposal for a new grading of moyamoya disease in adult patients. Cerebrovasc Dis (Basel, Switzerland) 32(1):41–50, PubMed PMID: 21576942. Epub 2011/05/18

    CAS  Google Scholar 

  67. Tanaka Y, Nariai T, Nagaoka T, Akimoto H, Ishiwata K, Ishii K et al (2006) Quantitative evaluation of cerebral hemodynamics in patients with moyamoya disease by dynamic susceptibility contrast magnetic resonance imaging–comparison with positron emission tomography. J Cereb Blood Flow Metab 26(2):291–300, PubMed PMID: 16049427. Epub 2005/07/29

    PubMed  Google Scholar 

  68. Ikezaki K, Matsushima T, Kuwabara Y, Suzuki SO, Nomura T, Fukui M (1994) Cerebral circulation and oxygen metabolism in childhood moyamoya disease: a perioperative positron emission tomography study. J Neurosurg 81(6):843–850, PubMed PMID: 7965114. Epub 1994/12/01

    CAS  PubMed  Google Scholar 

  69. Kanno I, Uemura K, Higano S, Murakami M, Iida H, Miura S et al (1988) Oxygen extraction fraction at maximally vasodilated tissue in the ischemic brain estimated from the regional CO2 responsiveness measured by positron emission tomography. J Cereb Blood Flow Metab 8(2):227–235, PubMed PMID: 3125186. Epub 1988/04/01

    CAS  PubMed  Google Scholar 

  70. Saito N, Nakagawara J, Nakamura H, Teramoto A (2004) Assessment of cerebral hemodynamics in childhood moyamoya disease using a quantitative and a semiquantitative IMP-SPECT study. Ann Nucl Med 18(4):323–331, PubMed PMID: 15359926. Epub 2004/09/14

    PubMed  Google Scholar 

  71. So Y, Lee HY, Kim SK, Lee JS, Wang KC, Cho BK et al (2005) Prediction of the clinical outcome of pediatric moyamoya disease with postoperative basal/acetazolamide stress brain perfusion SPECT after revascularization surgery. Stroke 36(7):1485–1489, PubMed PMID: 15947261. Epub 2005/06/11

    PubMed  Google Scholar 

  72. Touho H, Karasawa J, Ohnishi H (1996) Preoperative and postoperative evaluation of cerebral perfusion and vasodilatory capacity with 99mTc-HMPAO SPECT and acetazolamide in childhood Moyamoya disease. Stroke 27(2):282–289, PubMed PMID: 8571424. Epub 1996/02/01

    CAS  PubMed  Google Scholar 

  73. Iida H, Nakagawara J, Hatazawa J, Suzuki M, Takahashi M, Shimosegawa E et al (2012) Estimation of the arterial blood radioactivity concentration using the whole body-to-arterial blood partition coefficients and the cross-calibration factor in 123I-iodoamphetamine SPECT – towards a noninvasive clinical protocol with the QSPECT-DTARG method. Kaku Igaku 49(2):49–58, PubMed PMID: 22779347. Epub 2012/07/12

    PubMed  Google Scholar 

  74. Hatazawa J, Iida H, Shimosegawa E, Sato T, Murakami M, Miura Y (1997) Regional cerebral blood flow measurement with iodine-123-IMP autoradiography: normal values, reproducibility and sensitivity to hypoperfusion. J Nucl Med 38(7):1102–1108, PubMed PMID: 9225798. Epub 1997/07/01

    CAS  PubMed  Google Scholar 

  75. Iida H, Itoh H, Nakazawa M, Hatazawa J, Nishimura H, Onishi Y et al (1994) Quantitative mapping of regional cerebral blood flow using iodine-123-IMP and SPECT. J Nucl Med 35(12):2019–2030, PubMed PMID: 7989987. Epub 1994/12/01

    CAS  PubMed  Google Scholar 

  76. Kang KH, Kim HS, Kim SY (2008) Quantitative cerebrovascular reserve measured by acetazolamide-challenged dynamic CT perfusion in ischemic adult moyamoya disease: initial experience with angiographic correlation. AJNR Am J Neuroradiol 29(8):1487–1493, PubMed PMID: 18499785. Epub 2008/05/24

    CAS  PubMed  Google Scholar 

  77. Rim NJ, Kim HS, Shin YS, Kim SY (2008) Which CT perfusion parameter best reflects cerebrovascular reserve?: correlation of acetazolamide-challenged CT perfusion with single-photon emission CT in Moyamoya patients. AJNR Am J Neuroradiol 29(9):1658–1663, PubMed PMID: 18617583. Epub 2008/07/12

    PubMed  Google Scholar 

  78. Sakamoto S, Ohba S, Shibukawa M, Kiura Y, Arita K, Kurisu K (2006) CT perfusion imaging for childhood moyamoya disease before and after surgical revascularization. Acta Neurochir 148(1):77–81, discussion PubMed PMID: 16184319. Epub 2005/09/27

    CAS  PubMed  Google Scholar 

  79. Wintermark M, Maeder P, Verdun FR, Thiran JP, Valley JF, Schnyder P et al (2000) Using 80 kVp versus 120 kVp in perfusion CT measurement of regional cerebral blood flow. AJNR Am J Neuroradiol 21(10):1881–1884, PubMed PMID: 11110541. Epub 2000/12/08

    CAS  PubMed  Google Scholar 

  80. Kudo K, Christensen S, Sasaki M, Ostergaard L, Shirato H, Ogasawara K et al (2013) Accuracy and reliability assessment of CT and MR perfusion analysis software using a digital phantom. Radiology 267(1):201–211, PubMed PMID: 23220899. Pubmed Central PMCID: 3606546. Epub 2012/12/12

    PubMed Central  PubMed  Google Scholar 

  81. Zhang J, Wang J, Geng D, Li Y, Song D, Gu Y (2013) Whole-brain CT perfusion and CT angiography assessment of moyamoya disease before and after surgical revascularization: preliminary study with 256-slice CT. PLoS One 8(2):e57595, PubMed PMID: 23451248. Pubmed Central PMCID: 3579776. Epub 2013/03/02

    PubMed Central  CAS  PubMed  Google Scholar 

  82. Morkenborg J, Pedersen M, Jensen FT, Stodkilde-Jorgensen H, Djurhuus JC, Frokiaer J (2003) Quantitative assessment of Gd-DTPA contrast agent from signal enhancement: an in-vitro study. Magn Reson Imaging 21(6):637–643, PubMed PMID: 12915195. Epub 2003/08/14

    CAS  PubMed  Google Scholar 

  83. Nardone B, Saddleton E, Laumann AE, Edwards BJ, Raisch DW, McKoy JM et al (2014) Pediatric nephrogenic systemic fibrosis is rarely reported: a RADAR report. Pediatr Radiol 44(2):173–180, PubMed PMID: 24057195. Epub 2013/09/24

    PubMed Central  PubMed  Google Scholar 

  84. Detre JA, Subramanian VH, Mitchell MD, Smith DS, Kobayashi A, Zaman A et al (1990) Measurement of regional cerebral blood flow in cat brain using intracarotid 2H2O and 2H NMR imaging. Magn Reson Med 14(2):389–395, PubMed PMID: 2345518. Epub 1990/05/01

    CAS  PubMed  Google Scholar 

  85. Kuwabara Y, Ichiya Y, Sasaki M, Yoshida T, Masuda K, Matsushima T et al (1997) Response to hypercapnia in moyamoya disease. Cerebrovascular response to hypercapnia in pediatric and adult patients with moyamoya disease. Stroke 28(4):701–707, PubMed PMID: 9099182. Epub 1997/04/01

    CAS  PubMed  Google Scholar 

  86. Aoyama K, Fushimi Y, Okada T, Miyasaki A, Taki H, Shibamoto K et al (2012) Detection of symptomatic vasospasm after subarachnoid haemorrhage: initial findings from single time-point and serial measurements with arterial spin labelling. Eur Radiol 22(11):2382–2391, PubMed PMID: 22653285. Epub 2012/06/02

    PubMed  Google Scholar 

  87. Saida T, Masumoto T, Nakai Y, Shiigai M, Matsumura A, Minami M (2012) Moyamoya disease: evaluation of postoperative revascularization using multiphase selective arterial spin labeling MRI. J Comput Assist Tomogr 36(1):143–149, PubMed PMID: 22261785. Epub 2012/01/21. eng

    PubMed  Google Scholar 

  88. Noguchi T, Kawashima M, Irie H, Ootsuka T, Nishihara M, Matsushima T et al (2011) Arterial spin-labeling MR imaging in moyamoya disease compared with SPECT imaging. Eur J Radiol 80(3):e557–e562, PubMed PMID: 21315533. Epub 2011/02/15. eng

    PubMed  Google Scholar 

  89. Zaharchuk G, Do HM, Marks MP, Rosenberg J, Moseley ME, Steinberg GK (2011) Arterial spin-labeling MRI can identify the presence and intensity of collateral perfusion in patients with moyamoya disease. Stroke 42(9):2485–2491, PubMed PMID: 21799169. Pubmed Central PMCID: 3164217. Epub 2011/07/30. eng

    PubMed Central  PubMed  Google Scholar 

  90. Lee HY, Paeng JC, Lee DS, Lee JS, Oh CW, Cho MJ et al (2004) Efficacy assessment of cerebral arterial bypass surgery using statistical parametric mapping and probabilistic brain atlas on basal/acetazolamide brain perfusion SPECT. J Nucl Med 45(2):202–206, PubMed PMID: 14960636. Epub 2004/02/13. eng

    PubMed  Google Scholar 

  91. Sugino T, Mikami T, Miyata K, Suzuki K, Houkin K, Mikuni N (2013) Arterial spin-labeling magnetic resonance imaging after revascularization of moyamoya disease. J Stroke Cerebrovasc Dis 22(6):811–816, PubMed PMID: 22721824. Epub 2012/06/23

    PubMed  Google Scholar 

  92. Zhao WG, Luo Q, Jia JB, Yu JL (2013) Cerebral hyperperfusion syndrome after revascularization surgery in patients with moyamoya disease. Br J Neurosurg 27(3):321–325, PubMed PMID: 23461748. Epub 2013/03/07

    PubMed  Google Scholar 

  93. Hwang JW, Yang HM, Lee H, Lee HK, Jeon YT, Kim JE et al (2013) Predictive factors of symptomatic cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in adult patients with moyamoya disease. Br J Anaesth 110(5):773–779, PubMed PMID: 23274781. Epub 2013/01/01

    CAS  PubMed  Google Scholar 

  94. Ng J, Thompson D, Lumley JP, Saunders DE, Ganesan V (2012) Surgical revascularisation for childhood moyamoya. Child’s Nerv Syst 28(7):1041–1048, PubMed PMID: 22570164. Epub 2012/05/10

    CAS  Google Scholar 

  95. Cai X, Han S, Feske SK, Chou SH (2013) Pearls and oy-sters: small but consequential: intracerebral hemorrhage caused by lenticulostriate artery aneurysm. Neurology 80(9):e89–e91, PubMed PMID: 23439707. Pubmed Central PMCID: 3598457. Epub 2013/02/27

    PubMed Central  PubMed  Google Scholar 

  96. Gotoh K, Okada T, Satogami N, Yakami M, Takahashi JC, Yoshida K et al (2012) Evaluation of CT angiography for visualisation of the lenticulostriate artery: difference between normotensive and hypertensive patients. Br J Radiol 85(1019):e1004–e1008, PubMed PMID: 22744324. Pubmed Central PMCID: 3500797. Epub 2012/06/30

    PubMed Central  CAS  PubMed  Google Scholar 

  97. Gotoh K, Okada T, Miki Y, Ikedo M, Ninomiya A, Kamae T et al (2009) Visualization of the lenticulostriate artery with flow-sensitive black-blood acquisition in comparison with time-of-flight MR angiography. J Magn Reson Imaging 29(1):65–69, PubMed PMID: 19097113. Epub 2008/12/20

    PubMed  Google Scholar 

  98. Okuchi S, Okada T, Ihara M, Gotoh K, Kido A, Fujimoto K et al (2013) Visualization of lenticulostriate arteries by flow-sensitive black-blood MR angiography on a 1.5 T MRI system: a comparative study between subjects with and without stroke. AJNR Am J Neuroradiol 34(4):780–784, PubMed PMID: 23064597. Epub 2012/10/16

    CAS  PubMed  Google Scholar 

  99. Kimura T, Ikedo M, Takemoto S (2009) Hybrid of opposite-contrast MR angiography (HOP-MRA) combining time-of-flight and flow-sensitive black-blood contrasts. Magn Reson Med 62(2):450–458, PubMed PMID: 19526496. Epub 2009/06/16. eng

    PubMed  Google Scholar 

  100. Tsuchiya K, Kobayashi K, Nitatori T, Kimura T, Ikedo M, Takemoto S (2010) Hybrid of opposite-contrast MRA of the brain by combining time-of-flight and black-blood sequences: initial experience in major trunk stenoocclusive diseases. J Magn Reson Imaging 31(1):56–60, PubMed PMID: 20027573. Epub 2009/12/23. eng

    PubMed  Google Scholar 

  101. Miyamoto S, Akiyama Y, Nagata I, Karasawa J, Nozaki K, Hashimoto N et al (1998) Long-term outcome after STA-MCA anastomosis for moyamoya disease. Neurosurg Focus 5(5):e5, PubMed PMID: 17112208

    CAS  PubMed  Google Scholar 

  102. Nakagawara J, Takeda R, Suematsu K, Nakamura J (1997) Quantification of regional cerebral blood flow and vascular reserve in childhood moyamoya disease using [123I]IMP-ARG method. Clin Neurol Neurosurg 99(Suppl 2):S96–S99, PubMed PMID: 9409415. Epub 1997/12/31. eng

    PubMed  Google Scholar 

  103. Research Comittee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease)(2009) Research on intractable diseases of the Ministry of Health LaW, Japan. Recommendations for the Management of Moyamoya Disease. A Statement from Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease). Surg Cereb Stroke 37(5):321–337. Japanese

    Google Scholar 

  104. Karasawa J, Kikuchi H, Furuse S, Kawamura J, Sakaki T (1978) Treatment of moyamoya disease with STA-MCA anastomosis. J Neurosurg 49(5):679–688, PubMed PMID: 712390

    CAS  PubMed  Google Scholar 

  105. Miyamoto S, Kikuchi H, Karasawa J, Nagata I, Yamazoe N, Akiyama Y (1988) Pitfalls in the surgical treatment of moyamoya disease. Operative techniques for refractory cases. J Neurosurg 68(4):537–543, PubMed PMID: 3351581

    CAS  PubMed  Google Scholar 

  106. Karasawa J, Kikuchi H, Furuse S, Sakaki T, Yoshida Y (1977) A surgical treatment of “moyamoya” disease “encephalo-myo synangiosis”. Neurol Med Chir 17(1 Pt 1):29–37, PubMed PMID: 74029. Epub 1977/01/01. eng

    CAS  Google Scholar 

  107. Takeuchi S, Abe H, Ozawa T, Tanaka R (2000) Surgical treatment for moyamoya disease. Surgical effect of encephalo-galeo-synangiosis on moyamoya disease. Surg Cereb Stroke 28:98–103, Japanese

    Google Scholar 

  108. Matsushima Y, Fukai N, Tanaka K, Tsuruoka S, Inaba Y, Aoyagi M et al (1981) A new surgical treatment of moyamoya disease in children: a preliminary report. Surg Neurol 15(4):313–320, PubMed PMID: 7245020

    CAS  PubMed  Google Scholar 

  109. Karasawa J, Kikuchi H, Kawamura J, Sakai T (1980) Intracranial transplantation of the omentum for cerebrovascular moyamoya disease: a two-year follow-up study. Surg Neurol 14(6):444–449, PubMed PMID: 7221855

    CAS  PubMed  Google Scholar 

  110. Nakagawa Y, Abe H, Kamiyama H, Sawamura Y, Gotoh S, Kashiwaba T (1988) Revascularization surgery for 50 patients with moyamoya disease. In: Suzuki J (ed) Advances in surgery for cerebral stroke. Springer, Tokyo, pp 141–149

    Google Scholar 

  111. Fujimura M, Kaneta T, Mugikura S, Shimizu H, Tominaga T (2007) Temporary neurologic deterioration due to cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in patients with adult-onset moyamoya disease. Surg Neurol 67(3):273–282, PubMed PMID: 17320638. Epub 2007/02/27. eng

    PubMed  Google Scholar 

  112. Nishimoto A (1979) Moyamoya disease (author’s transl). Neurol Med Chir 19(3):221–228, PubMed PMID: 86959

    CAS  Google Scholar 

  113. Yoshida Y, Yoshimoto T, Shirane R, Sakurai Y (1999) Clinical course, surgical management, and long-term outcome of moyamoya patients with rebleeding after an episode of intracerebral hemorrhage: an extensive follow-up study. Stroke 30(11):2272–2276, PubMed PMID: 10548656

    CAS  PubMed  Google Scholar 

  114. Takahashi JC, Miyamoto S (2010) Moyamoya disease: recent progress and outlook. Neurol Med Chir 50(9):824–832, PubMed PMID: 20885116

    Google Scholar 

  115. Nishimoto A, Ueda K, Honma Y (eds) (1982) Follow-up study on outcome of the occlusion of the circle of Willis. Proceedings of the Research Committee on Spontaneous Occlusion of the Circle of Willis. Ministry of Health and Welfare, Tokyo

    Google Scholar 

  116. Kobayashi E, Saeki N, Oishi H, Hirai S, Yamaura A (2000) Long-term natural history of hemorrhagic moyamoya disease in 42 patients. J Neurosurg 93(6):976–980, PubMed PMID: 11117870

    CAS  PubMed  Google Scholar 

  117. Houkin K, Kamiyama H, Abe H, Takahashi A, Kuroda S (1996) Surgical therapy for adult moyamoya disease. Can surgical revascularization prevent the recurrence of intracerebral hemorrhage? Stroke 27(8):1342–1346, PubMed PMID: 8711799. Epub 1996/08/01. eng

    CAS  PubMed  Google Scholar 

  118. Kawaguchi S, Okuno S, Sakaki T (2000) Effect of direct arterial bypass on the prevention of future stroke in patients with the hemorrhagic variety of moyamoya disease. J Neurosurg 93(3):397–401, PubMed PMID: 10969936

    CAS  PubMed  Google Scholar 

  119. Fujii K, Ikezaki K, Irikura K, Miyasaka Y, Fukui M (1997) The efficacy of bypass surgery for the patients with hemorrhagic moyamoya disease. Clin Neurol Neurosurg 99(Suppl 2):S194–S195, PubMed PMID: 9409436

    PubMed  Google Scholar 

  120. Miyamoto S (2004) Study design for a prospective randomized trial of extracranial-intracranial bypass surgery for adults with moyamoya disease and hemorrhagic onset–the Japan Adult Moyamoya Trial Group. Neurol Med Chir 44(4):218–219, PubMed PMID: 15185765. Epub 2004/06/10. eng

    Google Scholar 

  121. Wiebers DO (1985) Ischemic cerebrovascular complications of pregnancy. Arch Neurol 42(11):1106–1113, PubMed PMID: 2864911

    CAS  PubMed  Google Scholar 

  122. Takahashi JC, Ikeda T, Iihara K, Miyamoto S (2012) Pregnancy and delivery in moyamoya disease: results of a nationwide survey in Japan. Neurol Med Chir 52(5):304–310, PubMed PMID: 22688067

    Google Scholar 

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Authors and Affiliations

  1. Department of Diagnostic Imaging and Nuclear Medicine, Graduate School of Medicine, Kyoto University, 54 Shogoin-Kawaharacho, Sakyo-ku, Kyoto, 606-8507, Japan

    Akira Yamamoto & Tomohisa Okada

  2. Department of Neurosurgery, National Cerebral and Cardiovascular Center, 5-7-1 Fujishirodai, Suita City, Osaka, Japan

    Jun C. Takahashi

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  1. Akira Yamamoto
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  2. Tomohisa Okada
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  3. Jun C. Takahashi
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Correspondence to Akira Yamamoto .

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  1. Department of Radiology, University of Cagliari, Cagliari, Italy

    Luca Saba

  2. Langone Medical Center and School of Med, New York University, New York, New York, USA

    Eytan Raz

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Yamamoto, A., Okada, T., Takahashi, J.C. (2014). Moyamoya Disease (Spontaneous Occlusion of the Circle of Willis). In: Saba, L., Raz, E. (eds) Neurovascular Imaging. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9212-2_25-1

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