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Moyamoya disease

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Abstract

The authors reviewed the Japanese literature on moyamoya disease. In the article we discuss the history of such investigations in Japan, the signs and symptoms, the diagnosis (especially concerning diagnostic criteria and magnetic resonance imaging), the pathology in relation to its etiology, and the current methods of treatment. On the whole, the main aim of the paper was to introduce our concept of moyamoya disease that is now current in Japan.

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References

  1. Asano S, Satoh T, Sakurai M, Yamamoto Y, Sadamoto K (1982) The advantage of coronal scanning in cerebral computed angiotomography for diagnosis of moyamoya disease. Radiology 145:709–711

    Google Scholar 

  2. Bodosi M, Merei FT, Gacs G (1981) Neurosurgical aspect of moyamoya disease. Zentralbl Neurochir 42:139–150

    Google Scholar 

  3. Brooks BS, Gammal TE, Adams RJ, Hartlage PL, Smith WB (1987) MR imaging of moyamoya in neurofibromatosis. AJNR 8:178–179

    Google Scholar 

  4. Chen YJ, Matsuo M, Tomita S, Kurokawa T, Ueda K (1985) Clinical usefulness of cerebral evocked potentials in moyamoya disease. Brain Dev 7:206

    Google Scholar 

  5. Coakham HB, Duchen LW, Scaravilli F (1979) Moyamoya disease. Clinical and radiological report of a case with associated myopathy. J Neurol Neurosurg Psychiatry 42:289–297

    Google Scholar 

  6. Cohen N, Berant M, Simon J (1980) Moyamoya and Fanconi's anemia. Pediatrics 65:804–805

    Google Scholar 

  7. Debran G, Lacour P (1974) A new case of moyamoya disease associated with several intracavernous aneurysms. Neuroradiology 7:277–282

    Google Scholar 

  8. Dhopesh VP, Dunn DP (1978) Moyamoya and Hageman factor (factor XII) deficiency. Arch Neurol 35:396

    Google Scholar 

  9. Ellison PH, Largent JA, Popp J (1981) Moyamoya disease associated with renal artery stenosis. Arch Neurol 38:467

    Google Scholar 

  10. Fujita K, Shirakuni T, Kojima N, Tamaki N, Matsumoto S (1986) Magnetic resonance imaging in moyamoya disease (in Japanese with English abstract). Neurosurgery (Tokyo) 14:324–330

    Google Scholar 

  11. Fukuyama Y, Umezu R (1979) A statistical analysis of obstraction of circle of Willis in children (in Japanese). Annual report of special working group of Welfare Ministry for moyamoya disease in fiscal year 1978, pp 25–29

  12. Fukuyama Y, Kuriya Y, Imezu R (1981) An experience of adrenocorticosteroid hormon in childhood occlusion of circle of Willis (in Japanese). Annual report of special working group of Welfare Ministry for moyamoya disease in fiscal year 1980, pp 45–51

  13. Furuse S, Matsumoto S, Tanaka Y, Andou S, Sawa H, Ishikawa S (1982) Moyamoya disease associated with a false aneurysm; Case report and review of the literature (with English abstract). Neurosurgery (Tokyo) 10:1005–1012

    Google Scholar 

  14. Handa H, Tani E, Kjikawa H, Satoh K, Yamashita J, Osaka K, Haebara H, Tamegai M, Kyogoku M (1969) Clinicopathological study on an adult case with cerebral arterial rete. (in Japanese with English abstract). Brain Nerve 21:77–87

    Google Scholar 

  15. Higashi K, Hatano M, Maza T (1974) Disease with abnormal intracranial vascular network complicated with intracerebral haematoma. J Neurol Neurosurg Psychiatry 37:365–369

    Google Scholar 

  16. Homma A, Tsutsui T, Kakunan N, Tobe H, Nagao S, Doi A, Nishimoto A (1981) Electroencephalographic study of moyamoya disease; relation of evocked potentials to clinical pictures (in Japanese). The annual report of special working group of Welfare Ministry for moyamoya disease in fiscal year 1980, pp 35–43

  17. Hosoda Y (1974) A pathomorphological analysis of so called “Spontaneous occlusion of circle of Willis” (cerebrovascular moyamoya disease) (in Japanese with English abstract). Brain Nerve 26:471–481

    Google Scholar 

  18. Hosoda Y, Ishii T, Mukai M, Yamada T, Kaburagi J, Nomura M, Ohtsuka K, Abe H, Tsuru M, Murase H (1979) A pathohistological study of 3 cases with abnormal vascular networks on cerebral angiography (in Japanese). Annual report of special working group of Welfare Ministry for moyamoya disease in fiscal year 1978, pp 116–131

  19. Hosoda Y, Ishii T, Ohta K, Murakami T, Furukaji K (1982) A study of obstraction of circle of Willis associated with vascular changes in other organs (in Japanese). Annual report of special working group of Welfare Ministry for moyamoya disease in fiscal year 1981, pp 145–153

  20. Ibayashi K, Takeuchi S, Ishii R, Tanaka R, Tsuruoka H (1985) Intelligence and memory function of juvenile patients with moyamoya disease (in Japanese with English abstract). Nerv Syst Child (Tokyo) 10:155–161

    Google Scholar 

  21. Ichiba N, Murakawa S, Ohtahara S (1984) A case of Down's syndrome with “moyamoya” disease; a consideration on fibromuscular dysplasia (in Japanese with English abstract) No To Hattatsu 16:487–491

    Google Scholar 

  22. Izawa M, Sentoh S (1981) A case of cerebrovascular moyamoya disease associated with basilar aneurysm. Neurosurgery (Tokyo) 9:365–370

    Google Scholar 

  23. Kamisasa A, Hiratsuka H, Inaba Y (1972) A case of an aneurysm arising in abnormal intracranial vascular networks (in Japanese with English abstract) Brain Nerve 24:463–468

    Google Scholar 

  24. Kaneko H, Okamura A, Nishida K, Watanabe M (1971) An autopsy case of intracranial rete mirabile (in Japanese with English abstract). Brain Nerve 23:1381–1387

    Google Scholar 

  25. Karasawa J, Kikuchi H, Furuse S, Sakaki T, Yoshida Y, Ohnishi H, Taki W (1977) Surgical treatment of “moyamoya” disease; “encephalo-myo synangiosis”. Neurol Med Chir (Tokyo) 17:29–37

    Google Scholar 

  26. Karasawa J, Kikuchi H, Furusawa S, Kawamura J, Sakaki T (1978) Treatment of moyamoya disease with STA-MCA anastomosis. J Neurosurg 49:679–688

    Google Scholar 

  27. Karasawa J, Kikuchi H, Fruse S (1980) Subependymal hemorrhage in moyamoya disease. Surg Neurol 13:118–120

    Google Scholar 

  28. Karasawa J, Kikuchi H, Kawamura J, Sakai T (1980) Intracranial transplantation of the omentum for cerebrovascular moyamoya disease; an two year follow up study. Surg Neurol 14:444–449

    Google Scholar 

  29. Karasawa J, Kikuchi H, Kuriyama Y, Sawada T, Kuro M, Kobayashi K, Koike T, Kitsugi MT (1981) Cerebral hemodynamics in “Moyamoya” disease; measurement of cerebral circulation and metabolism by use of the argon desaturation method in pre- and post-neurosurgical procedures (in Japanese with English abstract). Neurol Med Chir (Tokyo) 21:1161–1168

    Google Scholar 

  30. Karasawa J, Kikuchi H, Nagata I, Naruo Y, Ihara I, Nakagawara J, Miyamoto S, Hashimoto K, Kuriyama Y (1986) Cerebral hemodynamics in moyamoya disease (in Japanese with English abstract). Neurol Med Chir (Tokyo) 26:695–700

    Google Scholar 

  31. Kitahara T, Ariga N, Yamaura A, Makino H, Maki Y (1979) Familial occurrence of moyamoya disease: Report of three Japanese families. J Neurol Neurosurg Psychiatry 42:208–214

    Google Scholar 

  32. Kitahara T, Okunura K, Semba A, Yamaura A, Makino H (1982) Genetic and immunological analysis on moyamoya. J Neurol Neurosurg Psychiatry 45:1048–1052

    Google Scholar 

  33. Kobayashi K, Takeuchi S, Tsuchida T, Itoh J (1981) Encephalomyo-synangiosis in moyamoya disease; with special reference to postoperative angiography. Neurol Med Chir (Tokyo) 21:1229–1238

    Google Scholar 

  34. Kodama N (1971) The study of the aging of the perforating branches and its possibility of collateral pathway; concerning cerebrovascular “moyamoya” disease (in Japanese with English abstract). Brain Nerve 23:1389–1402

    Google Scholar 

  35. Kowada M, Momma F, Kikuchi K (1979) Intracranial aneurysm associated with cerebrovascular moyamoya disease: report of a case and review of 13 cases. Br J Radiol 52:236–237

    Google Scholar 

  36. Krayenbühl HA (1975) Moyamoya syndrome and the neurosurgeon. Surg Neurol 4:353–360

    Google Scholar 

  37. Kudou T (1971) Obstraction of circle of Willis; moyamoya disease (in Japanese). Int Med (Tokyo) 28:465–469

    Google Scholar 

  38. Lamas E, Robato RD, Cabello A, Abad JM (1978) Multiple intracranial occlusions (moyamoya disease) in patients with neurofibromatosis. One case report with autopsy. Acta Neurochir 45:133–145

    Google Scholar 

  39. Lee MLK, Cheung EMT (1973) Moyamoya disease as a cause of subarachnoid hemorrhage in Chinese. Brain 96:623–628

    Google Scholar 

  40. Maki Y (1976) What should we call this group of disease? (in Japanese with English abstract). Neurosurgery (Tokyo) 4:243–251

    Google Scholar 

  41. Maki Y, Nakada Y (1965) Autopsy case with angiomatous anomaly of basal internal carotid arteries (in Japanese). Brain Nerve 17:764–766

    Google Scholar 

  42. Maki Y, Yoshii Y (1980) Moyamoya disease (in Japanese). Clin Psychiatry 9:1337–1349

    Google Scholar 

  43. Maki Y, Horie T, Nakada Y (1968) Angiographical studies on the cases of obstructive disease with abnormal vascular network. J Chiba Med Soc 44:272–277

    Google Scholar 

  44. Maki Y, Nose T, Enomoto T, Tomono Y (1987) Moyamoya disease: can MRI predict the prognosis? 15th annual meeting of the international Society for Pediatric Neurosurgery

  45. Maki Y, Tomono Y, Nose T (1987) A study of periventricular high intensity on MRI of normal brain (in Japanese with English abstract). Annual report of special working group of Welfare Ministry in fiscal year 1986, pp 151–156

  46. Mathew NT, Abraham J, Chandy J (1970) Cerebral angiographic features in tuberculous meningitis. Neurology 20:1015–1023

    Google Scholar 

  47. Matsushima Y, Fukai N, Tanaka K, Tsuruoka S, Inaba Y, Aoyagi M, Ohno K (1981) A new surgical treatment of moyamoya disease in children: a preliminary report. Surg Neurol 15:313–320

    Google Scholar 

  48. Matsushima Y, Yamaguchi T, Takasato Y, Tomida S, Fukumoto T, Suzuki R, Tomita H, Inaba Y (1986) Changes in symptoms after encephalo-duro-arterio-synangiosis (EDAS) in pediatric moyamoya disease (in Japanese with English abstract). No To Hattatsu 18:3–7

    Google Scholar 

  49. Nagaya T, Takai H, Miyagi O, Tamada J (1982) Cerebral hemodynamics in cases with moyamoya disease (in Japanese with English abstract). Neurol Med Chir (Tokyo) 22:707–715

    Google Scholar 

  50. Nakada Y, Yoshii Y, Nose T, Tomono Y, Maki Y, Horie T (1978) Follow up of moyamoya in adult patients. Neurol Med Chir (Tokyo) 18:75–82

    Google Scholar 

  51. Nakahara S, Fujiwara J, Nakada Y (1987) Power spectral analysis of EEG basic rhythm in moyamoya disease in children. Brain Dev 9:192

    Google Scholar 

  52. Nakahara S, Kawashima K, Fujiwara J, Mtsumura A, Nakada Y (1984) EEG power spectra and gas analysis of arterial blood during and after hyperventilation in moyamoya disease in children. Brain Dev 6:218

    Google Scholar 

  53. Nishimoto A, Sugiu R (1964) Hemangiomatous malformation of bilateral carotid artery at the base of the brain. Proceedings of the 5th annual meeting of the Neuroradiological Association of Japan 5:2–9

    Google Scholar 

  54. Nomura R, Asai A, Hayashi M, Toda I, Imagawa K, Tochio H (1978) A study of CT, EEG, and clinical picture of moyamoya disease (in Japanese). Annual report of special working group of Welfare Ministry for moyamoya disease in fiscal year 1977, pp 32–40

  55. Nose T (1977) The diagnositic value of radioisotopic investigations in moyamoya (in Japanese with English abstract). Brain Nerve 29:611–619

    Google Scholar 

  56. Nishimoto A, Suzuki K, Homma A, Murota T (1984) Moyamoya disease in children (in Japanese with English abstract). Nerv Syst Child 9:189–197

    Google Scholar 

  57. Oka K, Yamashita M, Sadoshima S, Tanaka K (1981) Cerebral hemorrhage in moyamoya disease at autopsy. Virchows Arch [A] 392:247–261

    Google Scholar 

  58. Okamoto J, Mukai K, Kashiwara M, Ueda S, Matsumoto K (1982) A case of atypical moyamoya disease with a ruptured aneurysm on moyamoya vessel (in Japanese with English abstract). Neurosurgery (Tokyo) 10:897–903

    Google Scholar 

  59. Okuma A, Oshita H, Funakoshi T, Shikinami A, Yamada H (1980) A case of aneurysm in the cerebral moyamoya vessel (in Japanese with English abstract). Neurosurgery (Tokyo) 8:181–185

    Google Scholar 

  60. Olds MV, Griebel RW, Hoffman HJ, Craven M, Chuang S, Shutz H (1987) The surgical treatment of childhood moyamoya disease. J Neurosurg 66:675–680

    Google Scholar 

  61. Powarts D, Imbus C (1981) Cerebral vascular accidents in sickle cell anemia. Texas Rep Biol Med 1980–1981; 40:293–304

    Google Scholar 

  62. Sasaki T, Sakurai Y, Shimizu S, Ogawa A, Komatsu S, Onuma T, Suzuki J (1983) Bleeding pont in cerebral hemorrhage c in adults (with English abstract). Prog Comp Tomog 5:647–655

    Google Scholar 

  63. Sayama I, Fukazawa H, Yasui N, Suzuki A (1985) Child with moyamoya disease after by-pass surgery; Report of an autopsy case (with English abstract). Neurol Med Chir (Tokyo) 25:975–980

    Google Scholar 

  64. Serdaru M, Gray F, Merland J-J, Escourolle R, Grumback RG (1979) Moyamoya disease and intracerebral hematoma; Clinical pathological report. Neuroradiology 18:47–52

    Google Scholar 

  65. Shibata N, Mori K, Nakamura T (1985) Moyamoya disease in newborn; Case report (in Japanese with English abstract). Nerv Syst Child (Tokyo) 10:315–319

    Google Scholar 

  66. Shishido H, Tateno Y, Suda Y (1980) Cranial positron CT using 133NH3, and 11Co (in Japanese with English abstract). Nucl Med (Tokyo) 17:821–824

    Google Scholar 

  67. Sogaard I, Jorgensen J (1975) Familial occurrence of bilateral occlusion of internal carotid arteries moyamoya. Acta Neurochir (Wien) 31:245–252

    Google Scholar 

  68. Subirana A, Subirana M (1962) Malformations vasculaires du type de l'angiome arteriel racemeux. Rev Neurol (Paris) 107:545–550

    Google Scholar 

  69. Sunder TR (1981) Moyamoya disease in a patient with type I glycogenosis. Arch Neurol 38:251–253

    Google Scholar 

  70. Sunder TR, Erwin CW, Dubois PJ (1980) Hyperventilation induced abnormalities in the electroencephalogram of children eith moyamoya disease. Electroencephalogr Clin Neurophysiol 49:414–420

    Google Scholar 

  71. Suzuki J (1982) Moyamoya disease (in Japanese). No To Hattatsu (Tokyo) 14:4–16

    Google Scholar 

  72. Suzuki J, Takaku A (1968) The disease showing the abnormal vascular network at the base of brain particularly found in Japan (cerebro-vascular moyamoya disease) IV. Our opinion of dynamic change of these vascular network (in Japanese with English abstract). Brain Nerve 20:35–40

    Google Scholar 

  73. Suzuki J, Askira T, Asahi M, Kowada M (1965) Diseases showing the “fibrille” like vessels at the base of brain (in Japanese with English abstract). Brain Nerve 17:767–776

    Google Scholar 

  74. Suzuki J, Takaku A, Kodoma N, Sato S (1975) An attempt to treat cerebrovascular “moyamoya” disease in children. Child's Brain 1:193–206

    Google Scholar 

  75. Suzuki J, Kodama N, Fujiwara S, Kasai N, Yonemitsu T (1981) A study of etiology of moyamoya disease. Part I: Immune and sympathetic contribution to the cerebral arterial changes (in Japanese). Annual report of special working group of Welfare Ministry for moyamoya disease in fiscal year 1980, pp 21–34

  76. Tagawa T, Naritomi H, Mimaki T, Abe J, Onoe S, Ono J, Yabuuchi H, Sumi K, Kawada Y (1984) Mechanism of EEG abnormality caused by hyperventilation in children with moyamoya disease; Hemodynamic study by the 133Xe inhalation method. Brain Dev 6:218

    Google Scholar 

  77. Takahashi M, Saito Y, Konno K (1980) Intraventricular hemorrhage in childhood moyamoya disease. J CAT 4:117–120

    Google Scholar 

  78. Takeuchi K (1978) Moyamoya phenomenon and moyamoya disease (in Japanese with English abstract). Brain Nerve 30:1183–1191

    Google Scholar 

  79. Takeuchi K, Shimizu K (1957) Hypoplasia of the bilateral internal carotid arteries (in Japanese with English abstract). Brain Nerve 9:37–43

    Google Scholar 

  80. Takeyama E, Matsumori K, Sugimori T, Kagawa S, Fukuyama Y (1976) A case of anterior choroidal artery aneurysm combined with the abnormal intracranial vascular network (in Japanese with English abstract). Neurosurgery (Tokyo) 4:1075–1079

    Google Scholar 

  81. Tanaka Y, Takeuchi K, Ogashiwa M, Akai K, Omata K (1978) Moyamoya phenomenon conbined with cerebral anenrysm (in Japanese with English abstract). Brain Nerve 30:687–695

    Google Scholar 

  82. Tanoue K, Koyama T, Abe T, Nose T, Maki Y (1979) Agglutination of platelet in moyamoya (in Japanese). Int Med (Tokyo) 44:115–118

    Google Scholar 

  83. Tochio M, Kawasaki M, Imagawa K, Toda I, Hayashi M, Asai A, Momura R (1978) A comparative study of the Clinical features, computed tomography, and electroencephalic examination of moyamoya disease. Prog Comp Tomogr 1:257–271

    Google Scholar 

  84. Tohgemoto K, Hashimoto N, Maruoka Y, Knazawa Y, Asada M, Matsumoto S (1979) A valuability of measurement of plasma beta-thromboglobulin (beta-TG) for cerebrovascular diseases (in Japanese with English abstract). Brain Nerve 31:1277–1288

    Google Scholar 

  85. Tomida S, Matsushima Y, Yamazaki S, Itoh U, Inaba Y (1986) Five year follow up of two children of moyamoya disease after encephalo-duro-arterio-synangiosis (EDAS) operation (in Japanese with English abstract). No To Hattatsu 18:55–61

    Google Scholar 

  86. Umezu R (1983) Clinical picture and angiography of obstruction of circle of Willis in children with special reference to yearly changes (in Japanese). Jap J Pediatr 87:770–786

    Google Scholar 

  87. Yamaguchi T, Tashito M, Sugi T, Kitamura K (1980) Nation wide survey of obstraction of circle of Willis (in Japanese). Annual report of special working group of Welfare Ministry for moyamoya disease in fiscal year 1979, pp 9–16

  88. Yamano K, Onouchi Z, Shimada M (1974) Moyamoya disease and renal hypertension. A case probably caused by fibromuscular dysplasia (in Japanese with English abstract). No To Hattatsu: 12–16

  89. Yamashita M, Tanaka K, Matsuo T, Yokoyama K, Fujii T, Sakamoto H (1983) Cerebra; dissecting aneurysms in patient with moyamoya disease. Report of two cases. J Neurosurg 58:120–125

    Google Scholar 

  90. Yoshida T, Ikuta F, Ishii R, Tanaka R (1985) Small sacular aneurysms in moyamoya disease (in Japanese with English abstract). Neurol Med Chir (Tokyo) 25:95–102

    Google Scholar 

  91. Yoshii Y (1977) A study of prognosis of moyamoya: about clinical prognosis (in Japanese with English abstract). Brain Nerve 29:421–424

    Google Scholar 

  92. Yoshii Y (1977) Diagnostic value of CT scanning on moyamoya (in Japanese with English abstract). Brain Nerve 29:505–510

    Google Scholar 

  93. Yoshii N, Kudou T (1968) Electroencephalographical study on occlusion of the Willis arterial ring (in Japanese with English abstract). Clin Neurol (Tokyo) 8:301–309

    Google Scholar 

  94. Yuasa H, Tokito S, Izumi K, Hirabayashi K (1982) Cerebrovascular moyamoya disease associated with an intracranial pseudoaneurysm. Case report. J Neurosurg 56:131–134

    Google Scholar 

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  1. Department of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, Tennodai, 305, Tsukuba City, Ibaraki, Japan

    Yutaka Maki & Takao Enomoto

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  1. Yutaka Maki
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Maki, Y., Enomoto, T. Moyamoya disease. Child's Nerv Syst 4, 204–212 (1988). https://doi.org/10.1007/BF00270916

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