Moyamoya Disease

  • Kiyoshi Sato
  • Takeyoshi Shimoji
Part of the Principles of Pediatric Neurosurgery book series (PRINCPEDIATR)


At the fourteenth meeting of the Japanese Neurosurgical Society in 1955, Shimizu and Takeuchi reported a new disease entity designated as dysplasia of both internal carotid arteries1. This appears to be the first report of what we now refer to as moyamoya disease2. A succession of reports by a number of Japanese neurosurgeons3–6 of a similar disease followed, and the pathology of the disease was found to be characterized by the bilateral formation of abnormal netlike vessels at the base of the brain, associated with bilateral stenosis and/or occlusion of the arteries composing the circle of Willis.


Cerebral Blood Flow Internal Carotid Artery Middle Cerebral Artery Moyamoya Disease Superficial Temporal Artery 
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.


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  1. 1.
    Shimizu K, Takeuchi K: Dysplasia of the bilateral internal carotid arteries. Oral presentation at the 14th meeting of the Japan Neurosurgical Society, Wakayama, 1955.Google Scholar
  2. 2.
    Suzuki J, Takaku A: Cerebrovascular moyamoya disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20: 288 – 299, 1969.PubMedGoogle Scholar
  3. 3.
    Kudo T: Juvenile occlusion of the circle of Willis. Clin Neurology 5: 607 – 627, 1965.Google Scholar
  4. 4.
    Nishimoto A, Takeuchi S: Abnormal cerebrovascular network related to the internal carotid arteries. J Neurosurg 29: 255 – 260, 1968.PubMedCrossRefGoogle Scholar
  5. 5.
    Suzuki J, Takaku A, Asahi M, Kowada M: Disease showing the“Fibrille”-like vessels at the base of brain. No To Shinkei 17: 767 – 776, 1965.PubMedGoogle Scholar
  6. 6.
    TakeuchiK, Shimizu K: Hypoplasia of the bilateral internal carotid arteries. No To Shinkei 9: 37 – 43, 1957.Google Scholar
  7. 7.
    Kudo T: Spontaneous occlusion of the circle of Wills. A disease apparently confined to Japanese. Neurology 18: 485 – 496, 1968.PubMedGoogle Scholar
  8. 8.
    Weidner W, Hanafee W, Markham CH: Intracranial collateral circulation via leptomeningeal and rate mirabile anastomosis. Neurology 15: 39 – 48, 1965.PubMedGoogle Scholar
  9. 9.
    Krayenbuhl HA: The moyamoya syndrome and neurosurgeon. Surg Neurol 4: 353 – 360, 1975.PubMedGoogle Scholar
  10. 10.
    Taveras JM: Multiple progressive intracranial occlusions: A syndrome of children and young adults. AJR 106: 235 – 268, 1969.Google Scholar
  11. 11.
    Nishimoto A: Moyamoya disease. Neurol Med Chir 19: 221 – 228, 1979.CrossRefGoogle Scholar
  12. 12.
    Suzuki J, Kodama N: Moyamoya disease. A review. Stroke 14: 104 – 109, 1983.PubMedCrossRefGoogle Scholar
  13. 13.
    Adams HP Jr, Kassell NF, Wisoff HS, Drake CG: Intracranial saccular aneurysm and moyamoya disease. Stroke 10: 174 – 179, 1979.PubMedCrossRefGoogle Scholar
  14. 14.
    Nagamine Y, Takahashi S, Sonobe M: Multiple intracranial aneurysms associated with moyamoya disease. J Neurosurg 54: 673 – 676, 1981.PubMedCrossRefGoogle Scholar
  15. 15.
    Yamashita M, Oka K, Tanaka K: Histopathology of the brain Ovascular network in moyamoya disease. Stroke 14: 50 – 58, 1983.PubMedCrossRefGoogle Scholar
  16. 16.
    Fukuyama Y: Clinical and cerebral angiographic evolutions of idiopathic progress occlusive disease of the circle of Willis (“moyamoya” disease) in children. Brain Dev 7: 21 – 37, 1985.PubMedGoogle Scholar
  17. 17.
    Kitahara T, Ariga N, Yamaura A, Makino H, Maki Y: Familial occurrence of moyamoya disease: Report of three Japanese families. J Neurol Neurosurg Psychiatry, 42: 208 – 214, 1979.PubMedCrossRefGoogle Scholar
  18. 18.
    Kitahara T, Okumura K, Semba A, Yamaura A, Makino H: Genetic and immunologic analysis on moyamoya. J Neurol Neurosurg Psychiatry 45: 1048 – 1053, 1982.PubMedCrossRefGoogle Scholar
  19. 19.
    Nishimoto A, Takeuchi S: Moyamoya Disease. In: Vinken PJ, Bruyn GW (eds.) Handbook of Clinical Neurology. American Elsevier Publishing Company, New York, 1972, pp 352 – 382.Google Scholar
  20. 20.
    Miyamoto S, Kikuchi H, Karasawa J, Nagata I, Ikota T, Takeuchi S: Study of the posterior circulation in moyamoya disease. Clinical and Neuroradiological evaluation. J Neurosurg 61: 1032 – 1037, 1984.PubMedCrossRefGoogle Scholar
  21. 21.
    Sato S, Shibuya H, Matsushima Y, Suzuki S: Analysis of the angiographic findings in cases of childhood moyamoya disease. Neuroradiology 30: 111 – 119, 1988.CrossRefGoogle Scholar
  22. 22.
    Kodama N, Aoki Y, Hiraga H, Wada T, Suzuki J: Electroencephalographic findings in children with moyamoya disease. Arch Neurol 36: 16 – 19, 1979.PubMedGoogle Scholar
  23. 23.
    Takahashi A, Fujiwara S, Suzuki J: Cerebral angiography following hyperventilation in moyamoya disease. In reference to the “re-build up” phenomenon on EEG. No Shinkei Geka 13:255–264, 1985.Google Scholar
  24. 24.
    Ohyama H, Niizuma H, Fujiwara S, Suzuki J: EEG findings in moyamoya disease in children with special reference to the genesis of “re-build” up. No Shinkei Geka 13: 727 – 733, 1985.PubMedGoogle Scholar
  25. 25.
    Kameyama M, Shirane R, Tsurumi Y, Takahashi A, Fujiwara S, Suzuki J, Ito M, Ido T: Evaluation of cerebral blood flow and metabolism in childhood moyamoya disease. An investigation into “re-build-up” on EEG by positron CT. Childs Nerv Syst 2: 130 – 133, 1986.PubMedCrossRefGoogle Scholar
  26. 26.
    Sato H, Sato N, Tamaki N, Matsumoto S: Chronic low-perfusion state in children with moyamoya disease following revascularization. Childs Nerv Syst 6: 166 – 171, 1990.PubMedCrossRefGoogle Scholar
  27. 27.
    Takahashi M, Miyauchi T, Kowada M: Computed tomography of moyamoya disease: Demonstration of occluded arteries and collateral vessels as important diagnostic signs. Radiology 134: 671 – 676, 1980.PubMedGoogle Scholar
  28. 28.
    Takeuchi S, Kobayashi K, Tsuchida T, Imamura H, Tanaka R, Ito J: Computed tomography in moyamoya disease. J Comput Assist Tomogr 6: 24 – 32, 1982.PubMedCrossRefGoogle Scholar
  29. 29.
    Bruno A, Yuh WTC, Biller J, et al: Magnetic resonance imaging in young adults with cerebral infarction due to moyamoya. Arch Neurol 45: 303 – 306, 1988.PubMedGoogle Scholar
  30. 30.
    Fujisawa I, Asato R, Mishimura K, Kogashi K, Itoh K, et al: Moyamoya disease. MR imaging. Radiology 164: 103 – 105, 1987.PubMedGoogle Scholar
  31. 31.
    Fujita K, Shirakuni T, Kojima N, Matsumoto S: Magnetic resonance imaging. No Shinkei Geka 14: 324 – 330, 1986.PubMedGoogle Scholar
  32. 32.
    Rolak LA, Rokey R: Magnetic resonance imaging in moyamoya disease. J Child Neurol 1: 67 – 70, 1986.PubMedCrossRefGoogle Scholar
  33. 33.
    Ogawa A, Nakamura N, Sakurai Y, Kayama T, Wada T, Suzuki J: Cerebral blood flow in moyamoya disease. No To Shinkei 39: 199 – 203, 1987.PubMedGoogle Scholar
  34. 34.
    Takeuchi S, Tanaka R, Ishii R, Tsuchida T, Kobayashi K, Arai H: Cerebral hemodynamics in patients with moyamoya disease. A study of regional cerebral blood flow by the 133Xe inhalation method. Surg-Neurol 23: 468 – 474, 1985.PubMedCrossRefGoogle Scholar
  35. 35.
    Nakao K, Yamada K, Hayakawa T, Tagawa T, Yoshimine T, Ushio Y, Mogami H: Intraoperative measurement of cortical blood flow and its C02 response in childhood moyamoya disease. Neurosurgery 21: 509 – 14, 1987.PubMedCrossRefGoogle Scholar
  36. 36.
    Tatemichi TK, Prohovink I, Mohr JP, Correll JW, Quest DO, Jarvis I: Reduced hypercapnic vasoreactivity in moyamoya disease. Neurology 38: 1575 – 1581, 1988.PubMedGoogle Scholar
  37. 37.
    Taki W, Yonekawa Y, Kobayashi A, Ishikawa M, Kikuchi H, Nishizawa S, Senda M, Yonekawa Y, Fukuyama H, Harada K, et al: Cerebral circulation and oxygen metabolism in moyamoya disease of ischemic type in children. Childs Nerv Syst 4: 259 – 262, 1988.PubMedGoogle Scholar
  38. 37a.
    Tomura N, Kanno I, Shishido F, Higano S, Fujita H, Inugami A, Tabata K, Uemura K, Sayama I, Yasui N: Vascular responses in cerebrovascular “Moyamoya” disease evaluated by positron emission tomography. No To Shinkei 41: 895 – 904, 1989.PubMedGoogle Scholar
  39. 38.
    Kuwabara Y, Ichiya Y, Otsuka M, Tahara T, Gunasekera R, Hasuo K, Masuda K, Matsushima T, Fukui M: Cerebral hemodynamic change in the child and the adult with moyamoya disease. Stroke 21: 272 – 277, 1990.PubMedCrossRefGoogle Scholar
  40. 39.
    Nishimoto A, Takeuchi S: Moyamoya Disease. In: Vinken PJ, Bruyn GW (eds.) Handbook of Clinical Neurology, American Elsevier Publishing Company, New York, 1972, pp 352 – 383.Google Scholar
  41. 40.
    Ohtoh T, Iwasaki Y, Namiki T, Nakamura K, Sakurai Y, Ogawa A, Wada T: Hemodynamic characteristics of the vertebrobasilar system in moyamoya disease. A histometric study. Hum- Pathol 19: 465 – 470, 1988.PubMedCrossRefGoogle Scholar
  42. 41.
    Oka K, Yamashita M, Sadoshima S, Tanaka K: Cerebral hemorrhage in moyamoya disease at autopsy. Virchows Arch Pathol Anat 392: 247 – 261, 1981.PubMedGoogle Scholar
  43. 42.
    Gomez CR, Hogan PA: Unilateral Moyamoya disease in an adult. A case history. Angiology 38: 342 – 346, 1987.PubMedCrossRefGoogle Scholar
  44. 43.
    Matsushima T, Fukui M, Fujii K, Fujiwara S, Nagata S, Kitamura K, Kuwabara Y: Two pediatric cases with occlusions of the ipsilateral internal carotid and posterior cerebral arteries associated with moyamoya vessels. “Unilateral” moyamoya disease. Surg Neurol 33: 276 – 280, 1990.PubMedCrossRefGoogle Scholar
  45. 44.
    Matsushima T, Take S, Fujii K, Fukui M, Hasuo K, Kuwabara Y, Kitamura K: A case of moyamoya disease with progressive involvement from unilateral to bilateral. Surg Neurol 30: 471 – 475, 1988.PubMedCrossRefGoogle Scholar
  46. 45.
    Takeuchi K: Moyamoya syndrome and Moyamoya disease. No To Shinkei 30: 1183 – 1191, 1978.PubMedGoogle Scholar
  47. 46.
    Wilms G, Marchal G, Van Fraeyenhoven L, Demaerel P, Casaer P, Van Elderen S, Baert AL: Unilateral moyamoya disease. MRI findings. Neuroradiology 31: 442, 1989.PubMedCrossRefGoogle Scholar
  48. 47.
    Ishikawa M, Handa H, Mori K, Matsuda I: Moyamoya vessels on the tumor in the seller region. Arch Jap Chir 48: 639 – 644, 1979.Google Scholar
  49. 48.
    Tsuji N, Kuriyama T, Iwamoto M, Shizuki K: Moyamoya disease associated with craniopharyngioma. Surg Neurol 21: 588 – 592, 1984.PubMedCrossRefGoogle Scholar
  50. 49.
    Hilar SK, Solomon GE, Gold AP, Carter S: Primary cerebral arterial occlusive disease in children. Part II. Neurocutaneous syndromes. Radiology 99: 87 – 94, 1974.Google Scholar
  51. 50.
    Yashon D, Johnson AB, Jane JA: Bilateral internal carotid artery occlusion secondary to closed head injuries. J Neurol Neurosurg Psychiatry 27: 547 – 552, 1964.PubMedCrossRefGoogle Scholar
  52. 51.
    Pilz P: Fibromuscular dysplasia and multiple dissecting aneurysms of intracranial arteries. A further cause of moyamoya syndrome. Stroke 7: 393 – 398, 1976.PubMedCrossRefGoogle Scholar
  53. 52.
    Bingham WFB, Beguin EA, Ramirez-Lassepas: Moyamoya disease in pregnancy. Wis Med J 79: 21 – 25, 1980.PubMedGoogle Scholar
  54. 53.
    Rajakulasingam K, Cerullo LJ, Raimondi AJ: Childhood moyamoya syndrome. Childs Brain 5: 467 – 475, 1979.PubMedGoogle Scholar
  55. 54.
    Kurokawa T, Tomita S, Ueda K, Narazaki O, Hanai T, Hasuo K, Matsushima T, Kitamura K: Prognosis of occlusive disease of the circle of Willis (moyamoya disease) in children. Pediatr Neurol 1: 274 – 277, 1985.PubMedCrossRefGoogle Scholar
  56. 55.
    Tsubokawa T, Kikuchi M, Asano S, Itoh H, Urabe M: Surgical treatment for intracranial thrombosis. Case report of “Durapexia”. Neurol Med Chir 6: 428 – 429, 1964.CrossRefGoogle Scholar
  57. 56.
    Suzuki J, Takaku A, Kodama N, Sato S: An attempt to treat cerebrovascular moyamoya disease in children. Childs Brain. 1: 193 – 206, 1975.PubMedGoogle Scholar
  58. 57.
    Karasawa J, Kikuchi H, Furuse S: A surgical treatment of “moyamoya” disease “encephalo-myo-synangiosis”. Neurol Med Chir 17: 30 – 37, 1977.Google Scholar
  59. 58.
    Karasawa J, Kikuchi H, Furuse S, Kawamura J, Sasaki T: Treatment of moyamoya disease with STA-MCA anastomosis. J Neurosurg 49: 679 – 688, 1978.PubMedCrossRefGoogle Scholar
  60. 59.
    Takeuchi S, Kikuchi H, Karasawa J, Yamagata S, Nagata I: Regional cortical flow during extra-intracranial bypass surgery in young patients with moyamoya disease. Neuro Med Chir 29: 10 – 14, 1989.CrossRefGoogle Scholar
  61. 60.
    Amine ARC, Moody RA, Meeks W: Bilateral temporal middle cerebral artery anastomosis for moyamoya syndrome. Surg Neurol 8: 3 – 6, 1977.PubMedGoogle Scholar
  62. 61.
    Karasawa J, Kikuchi H, Kawamura J, Sakaki T: Intracranial transplantation of the omentum for cerebrovascular moyamoya disease. A two year follow-up study. Surg Neurol 14: 444 – 449, 1980.PubMedGoogle Scholar
  63. 62.
    Matsushima Y, Fukai N, Tanaka K, Tsuruoka S, Inaba Y, Aoyagi M, Ohno K: A new surgical treatment of moyamoya disease in children. A preliminary report. Surg Neurol 15: 313 – 320, 1981.PubMedCrossRefGoogle Scholar
  64. 63.
    Ichikawa A, Tanaka R, Takeuchi S, Koike T, Ishii R: Reconstructive vascular surgery in children with moyamoya disease. Neurol Med Chir 29: 106 – 112, 1989.CrossRefGoogle Scholar
  65. 64.
    Nakagawa Y, Gotoh H, Shimoyama M, Ohtsuka K, Mabuchi S, Sawamura Y, Abe H, Tsuru M: Reconstructive operation for moyamoya disease. Surgical indication for the hemorrhagic type, and preferable operative methods. Neurol Med Chir 23: 464 – 470, 1983.CrossRefGoogle Scholar
  66. 65.
    Matsushima Y, Inaha Y: Moyamoya disease in children and its surgical treatment. The introduction of a new surgical procedure and its follow up angiograms. Childs Brain 11: 155 – 170, 1984.PubMedGoogle Scholar
  67. 66.
    Olds MV, Griebel RW, Hoffman HJ, Craven M, Chuang S, Schutz H: The surgical treatment of childhood moyamoya disease. J Neurosurg 66: 675 – 680, 1987.PubMedCrossRefGoogle Scholar
  68. 67.
    Matsushima T, Fujiwara S, Nagata S, Fujii K, Fukui M, Kitamura K, Hasuo K: Surgical treatment for paediatric patients with moyamoya disease by indirect revascularization procedures (EDAS, EMS, EMAS). Acta Neurochir 98: 135 – 140, 1989.CrossRefGoogle Scholar
  69. 68.
    Fujita K, Tamaki N, Matsumoto S: Surgical treatment of moyamoya disease in children: which is more effective procedure. DEAS or EMS? Childs Nerv Syst 2: 134 – 138, 1986.PubMedCrossRefGoogle Scholar
  70. 69.
    Eller TW, Pasternak JF: Revascularization for moyamoya disease. Five-year follow-up. Surg Neurol 28: 463 – 467, 1987.PubMedCrossRefGoogle Scholar
  71. 70.
    Matsushima Y, Inaba Y: The specificity of the collaterals to the brain through the study and treatment of moyamoya disease. Stroke 17: 117 – 122, 1986.PubMedCrossRefGoogle Scholar
  72. 71.
    Shimoji T, Sato K, Ito M: Cerebral revascularization for moyamoya disease. Usefulness of EDAS Nervous System in Children. 15: 161 – 168, 1990.Google Scholar
  73. 72.
    Miyamoto S, Kikuchi H, Karasawa J, Nagata I, Yamazoe N, Akiyama Y: Pitfalls in the surgical treatment of moyamoya disease Operative techniques for refractory cases. J Neurosurg 68: 537 – 543, 1973.Google Scholar
  74. 73.
    Ishii R, Takeuchi S, Kobayashi K, Tanaka R: Intelligence in children with moyamoya disease. Evaluation after surgical treatments with special reference to changes in cerebral blood flow. Stroke 15: 873 – 877, 1984.PubMedCrossRefGoogle Scholar
  75. 74.
    Takeuchi S, Tsuchida T, Kobayashi K, Fukuda M, Ishii R, Tanaka R, Ito J: Treatment of moyamoya disease by temporal muscle graft encephalo myo synangiosis. Childs Brain 10: 1 – 15, 1983.PubMedGoogle Scholar
  76. 75.
    Suzuki R, Matsushima Y, Takada Y, Nariai T, Wakabayashi S, Tone O: Changes in cerebral hemodynamics following encephalo-duro-arterio-synangiosis (EDAS). In young patients with moyamoya disease. Surg Neurol 31:343–349, 1989.Google Scholar
  77. 76.
    Moritake K, Handa H, Yonekawa Y, Taki W, Okuno T: Follow-up study on the relationship between age at onset of illness and outcome in patients with moyamoya disease. No Shinkei Geka 14: 957 – 963, 1986.PubMedGoogle Scholar
  78. 77.
    Bingham RM, Wilkinson DJ: Anaesthetic management in moyamoya disease. Anaesthesia 40: 1198–1202 (68), 1985.Google Scholar

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© Springer-Verlag New York Inc. 1992

Authors and Affiliations

  • Kiyoshi Sato
  • Takeyoshi Shimoji

There are no affiliations available

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