Neurological Surgery 脳神経外科40巻1号

2012年01月発行

連載 脳神経外科手術手技に関する私見とその歴史的背景

9．モヤモヤ病

著者： 米川泰弘

所属機関：

ページ範囲：P.67 - P.87

Ⅰ．はじめに

　過日（2011年7月21～22日），ここKinderspital Zürichで第2回のMoyamoya disease （MMD）のInternational Meetingが開催された（Fig. 1）．第1回は米国のStanford大学（Prof.Steinberg）で2009年に開催されたという．今回の開催責任者はFrau PD Dr.Khanである．彼女は私の在任中にMoyamoya angiopathy（MMA）をテーマにPrivatdozent（PD）（私講師：教授に推挙される前提資格）の論文を仕上げ，念願のPDとなったが，私の2007年の定年退官を機にZürichを出立し，Lausanne，Stanfordと遍歴を重ね，Kinderspital Zürichに帰ってきたのである．私は具体的なことにタッチせずhonorary lectureのみの責ということで，気安く参加した．

　ちなみにKinderspital Zürichは，私どもの学生時代，1960年代前半に小児科学の教科書として先輩から推薦されていた「FanconiのKinderheilkunde」（Fig. 2）として有名であったProf.Fanconiが在任した病院である．当時は，小児科のよい日本語の教科書がまだなかった時代で，ドイツ語の本書が学生には薦められていた［ちなみに，当時，解剖学ではRauber-Kopsch（ドイツ語），生理学ではFulton（英語），内科学ではHarrison（英語）が薦められていた］．また，私が，Zürich大学病院脳神経外科の助手時代の1970年代に小児のV-A shunt，V-P shuntを行いに出かけた病院で，のちにProf.Rickham（Rickham reservoirで有名）が英国から病院長として招聘され，10年ほど前まで在任された名門小児病院である．ちなみに，Fanconi's anemiaは本症と結合して，Moyamoya syndrome（MMS）を形成することもあり得る．

　日本からは，北海道大学の寳金清博教授とその一門，東北大学，それに福井大学の菊田健一郎教授らの参加があった．また，米国からはProf.Scott（Boston），Prof.Steinberg（Stanford）らの一門，それにドイツ，フランス，イギリスから脳神経外科，神経内科，molecular biologyの研究者の参加があった．Bypass surgeryで一世を風靡したReichman，Chater，Peerless，Ausman，Fein，Khodadad，Schmiedek，Gratzlなどのbypass surgeonの姿，顔はなく，講演でも言及されることはなかった．時代が過ぎてゆくのを感じた．

　私は基調講演でMMDの発見，厚生省難病調査研究班研究の成果をもとに治療，epidemiology，pathophysiology，病因究明の歴史と本症のupdateを述べたが，以下の内容は，そのoutlineと，このmeetingで感じた事柄である．今回は，私にとっても，これらの発展の歴史を辿りなおし，自分の行っている臨床を考えるよい機会であった．日本で，1992年末までに約70症例，Zürichで1993年以来同じく70症例ほどの血流再建術を行ったが，その結果は別の論文にゆずるとして，私が今回この機会に，様々な人々の助けも得ながら文献などを掘り出し，自分の経験をもとに見直した知見を以下に述べるが，興味のある人はさらに自分で追究していただきたい．

参考文献

1）Baba T, Houkin K, Kuroda S：Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 79：900-904, 2008

2）Donaghy RM, Yaşargil G：Extracranial blood flow diversions in microvascular surgery. Paper presented at the 36th annual meeting of the American Association of Neurological Surgeons, Chicago, 1968

3）Fodstad H, Bodosi M, Fossell A, Perricone D：Moyamoya disease in patients of Finno-Ugric origin. Br J Neurosurg 10：179-186, 1996

4）Fujimura M, Kaneta T, Mugikura S, Shimizu H, Tominaga T：Temporary neurologic deterioration due to cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in patients with adult-onset moyamoya disease. Surg Neurol 67：273-282, 2007

5）Fukui M；Members of Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan：Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (“moyamoya” disease). Clin Neurol Neurosurg 99 (suppl 2)：S238-S240, 1997

6）Golby AJ, Marks MP, Thompson RG, Steinberg GK：Direct and combined revascularization in pediatric Moyamoya disease. Neurosurgery 45：50-60, 1999

7）Goto Y, Yonekawa Y：Worldwide distribution of moyamoya disease. Neurol Med Chir (Tokyo) 32：883-886, 1992

8）Handa H, Yonekawa Y：Analysis of filing data bank of 1500 cases of spontaneous occlusion of the circle of Willis and study of 200 cases for more than 5 years. Stroke (Tokyo) 7：477-480, 1985

9）Henschen C：Operative Revascularization des zirkulatorisch geschadigten Gehirns durch Auflage gestielten Muskellappen (Encephalo-myo-synangiose). Langenbecks Arch Klein Chir 264：392-401, 1950

10）Hoshimaru M, Takahashi JA, Kikuchi H, Nagata I, Hatanaka M：Possible role of fibroblastic growth factor in the pathogenesis of moyamoya disease. An immunohistochemical study. J Neurosurg 75：267-270, 1991

11）Hosoda Y：Pathology of so-called “spontaneous occlusion of the circle of Willis”. Pathol Annu 19：221-244, 1984

12）Ikeda E, Hosoda Y：Distribution of thrombotic lesions in the cerebral arteries in spontaneous occlusion of the circle of Willis：cerebrovascular moyamoya disease. Clin Neuropathol 12：44-48, 1993

13）Iwama T, Hashimoto N, Yonekawa Y：The relevance of hemodynamic factors in perioperative complications in childhood moyamoya disease. Clinical studies. Neurosurgery 38：1120-1126, 1996

14）Iwama T, Hashimoto N, Murai BN：Intracranial rebleeding in Moyamoya disease. J Clin Neurosci 4：169-172, 1997

15）Iwama T, Hashimoto N, Miyake H, Yonekawa Y：Direct revascularization to the anterior cerebral artery territory in patients with moyamoya disease. Report of five cases. Neurosurgery 42：1157-1161, 1998

16）Karasawa J, Kikuchi H, Furuse S：A surgical treatment of moyamoya disease. Encephalomyosynangiosis. Neurol Med Chir (Tokyo) 17：29-37, 1977

17）Karasawa J, Kikuchi H, Furuse S, Kawamura J, Sasaki T：Treatment of moyamoya disease with STA-MCA anastomosis. J Neurosurg 49：679-688, 1978

18）Karasawa J, Touho H, Ohnishi H, Miyamoto S, Kikuchi H：Long-term follow-up study after extracranial-intracranial bypass surgery for anterior circulation ischemia in childhood moyamoya disease. J Neurosurg 77：84-89, 1992

19）Karasawa J, Touho H, Ohnishi H, Miyamoto S, Kikuchi H：Cerebral vascularization using omental transplantation for childhood moyamoya disease. J Neurosurg 79：192-196, 1993

20）Kawaguchi S, Sakaki T, Kakizaki T, Kamada K, Shimomura T, Iwanaga H：Clinical features of the haemorrhage type moyamoya disease based on 31 cases. Acta Neurochir (Wien) 138：1200-1210, 1996

21）Kawaguchi S, Sakaki T, Morimoto T, Kakizaki T, Kamada K：Characteristics of intracranial aneurysms associated with moyamoya disease. A review of 111 cases. Acta Neurochir (Wien) 138：1287-1294, 1996

22）Kawano T, Fukui M, Hashimoto N, Yonekawa Y：Follow-up study of patients with “unilateral” moyamoya disease. Neurol Med Chir (Tokyo) 34：744-747, 1994

23）Khan N, Schuknecht B, Boltshauser E, Capone A, Buck A, Imhof HG, Yonekawa Y：Moyamoya disease and moyamoya syndrome：Experience in Europe：Choice of revascularisation procedures. Acta Neurochir (Wien) 145：1061-1071, 2003

24）Kikuta K, Takagi Y, Fushimi Y, Ishizu K, Okada T, Hanakawa T, Miki Y, Fukuyama H, Nozaki K, Hashimoto N：“Target bypass”：a method for preoperative targeting of a recipient artery in superficial temporal artery -to. -middle cerebral artery anastomoses. Neurosurgery 59 (ONS 4 suppl 2) ONS320-327, 2006

25）Kikuta K, Takagi Y, Nozaki K, Sawamoto N, Fukuyama H, Hashimoto N：The presence of multiple microbleeds as a predictor of subsequent cerebral haemorrhage in patients with Moyamoya disease. Neurosurgery 62：104-112, 2008

26）Krayenbühl HA：The Moyamoya syndrome and the neurosurgeon. Surg Neurol 4：353-360, 1975

27）Kudo T：Spontaneous occlusion of the circle of Willis：a disease apparently confined to Japanese. Neurology 18：485-496, 1968

28）Kuroda S, Ishikawa T, Houkin K, Nanba R, Hokari M, Iwasaki Y：Incidence and clinical features of disease progression in adult moyamoya disease. Stroke 36：2148-2153, 2005

29）Kuroda S, Hashimoto N, Yoshimoto T, Iwasaki Y；for the Research Committee on Moyamoya Disease in Japan：Radiological findings, clinical course, and outcome in asymptomatic moyamoya disease：results of multicenter survey in Japan. Stroke 38：1430-1435, 2007

30）Kwon OK, Kim SH：Endovascular treatment of Moyamoya disease. In Cho BK, Tominaga T (eds)：Moyamoya disease update. Springer, Tokyo, 2010, pp252-260

31）Lasjaunias PL：Segmental identity and vulnerability in cerebral arteries. Interv Neuroradiol 6：113-124, 2000

32）牧　豊，中田義隆：脳底部内頸動脈血管腫奇形の剖検例について．No To Shinkei 17：764-766, 1965

33）Mineharu Y, Liu W, Inoue K, Matsuura N, Inoue S, Takenaka K, Ikeda H, Houkin K, Takagi Y, Kikuta K, Nozaki K, Hashimoto N, Koizumi A：Autosomal dominant moyamoya disease maps to chromosome 17q25.3. Neurology 70：2357-2363, 2008

34）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

35）Miyamoto S, Japan Adult Moyamoya Trial Group：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 (Tokyo) 44：218-219, 2004

36）Muroi C, Yonekawa Y, Khan N, Pangalu A, Keller E：Case report. Metabolic changes after H215O-positron emission tomography with acetazolamide in a patient with moyamoya disease：case report and review of previous cases. J Neurosurg Anesthesiol 15：131-139, 2003

37）Muroi C, Khan N, Bellut D, Fujioka M, Yonekawa Y：Extracranial-intracranial bypass in atherosclerotic cerebrovascular disease：report of a single centre experience. Br J Nuerosurg 25：357-362, 2011

38）Nariai T, Matsushima Y, Imae S, Tanaka Y, Ishii K, Senda M, Ohno K：Severe haemodynamic stress in selected subtypes of patients with moyamoya disease：a positron emission tomography study. J Neurol Neurosurg Psychiatry 76：663-669, 2005

39）Nishimoto A, Takeuchi S：Abnormal cerebro-vascular network related to the internal carotid arteries. J Neurosurg 29：255-260, 1968

40）Peerless SJ：Risk factors of moyamoya disease in Canada and the USA. Clin Neurol Neurosurg 99 (suppl 2)：45-48, 1997

41）Picard L, Lévesque M, Crouzet G, Simon J, Andre JM：Le syndrome ≪Moyamoya≫. J Neuroradiol 1：47-54, 1974

42）Reichman OH, Anderson RE, Roberts TS, Heilbrun MP：The treatment of intracranial occlusive cerebrovascular disease by STA-cortical MCA anastomosis. In Handa H (ed)：Microneurosurgery. Igaku Shoin, Tokyo, 1975, pp31-46

43）Sakamoto S, Kiura Y, Yamasaki F, Shibukawa M, Ohba S, Shrestha P, Sugiyama K, Kurisu K：Expression of vascular endothelial growth factor in dura mater of patients with moyamoya disease. Neurosurg Rev 31：77-81, 2007

44）Sato K, Shirane R, Yoshimoto T：Perioperative factors related to the development of ischemic complications in patients with moyamoya disease. Childs Nerv Syst 13：68-72, 1997

45）Suzuki J, Takaku A：Cerebrovascular “moyamoya” disease：Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20：288-299, 1969

46）Suzuki J, Kodama N：Moyamoya disease ― a review. Stroke 14：104-109, 1983

47）Takagi Y, Kikuta K, Sadamasa N, Nozaki K, Hashimoto N：Caspase-3-dependent apoptosis in middle cerebral arteries in patients with moyamoya disease. Neurosurgery 59：894-901, 2006

48）竹内一夫，清水健太郎：両側内頚動脈形成不全症．No To Shinkei 9：37-43, 1957

49）Taki Y, 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

50）田中公人，米川泰弘，河野寛幸，片桐邦彦，佐藤耕造：Vein Graftを使用した閉塞性前大脳動脈に対する血行再建術の2例．No Shinkei Geka 20：171-176, 1992

51）Uchino K, Johnston SC, Becker KJ, Tirschwell DL：Moyamoya disease in Washington state and Califormnia. Neurology 65：956-958, 2005

52）Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, Kojima M, Lin Y, Ohno Y：Epidemiological features of moyamoya disease in Japan：findings from a nationwide survey. Clin Neurol Neurosurg 99 (suppl 2)：S1-S5, 1997

53）Weidner W, Hanafee W, Markham CH：Intracranial collateral circulation via leptomeningeal and rete mirabile anastomoses. Neurology 15：39-48, 1965

54）Yamashita M, Oka K, Tanaka K：Histopathology of the brain vascular network in Moyamoya disease. Stroke 14：50-58, 1983

55）Yaşargil MG：Microsurgery Applied to Neurosurgery. Thieme, Stuttgart, 1969

56）Yonekawa Y, Yaşargil MG：Arterial extracranial intracranial anastomosis：Technical and clinical aspects：results. In Krayenbuhl H (ed)：Advances and Technical Standards of Neurosurgery. Vol 3. Springer, Vienna, 1976, pp47-78

57）Yonekawa Y, Yaşargil MG：Brain vascularization by transplanted omentum. A possible treatment of cerebral ischemia. Neurosurgery 1：256-259, 1977

58）Yonekawa Y, Handa H, Moritake K, Taki W, Miyake H, Kobayashi A, Asato R, Hoshimaru M：Revascularization in children with Moyamoya disease：Low-density areas and regional cerebral blood flow after operation. In Handa H, Kikuchi H, Yonekawa Y (eds)：Microvascular anstomoses for cerebral ischemia. Igaku shoin, New York-Tokyo, 1985, pp272-274

59）Yonekawa Y, Yamashita K, Taki W, Kikuchi H：Clinical features of hemorrhagic type of Moyamoya disease-Special emphasis on cases with rebleeding-Annual Report 1988 of the research committee of spontaneous occlusion of the circle of Willis, pp81-84

60）Yonekawa Y, Goto Y, Ogata N：Moyamoya disease：Diagnosis, treatment, and recent achievement. Part Ⅳ. Specific medical disease and stroke. In Barnett HJM, Mohr JP, Stein BM, et al (eds)：Stroke, Pathophysiology, Diagnosis and Management Churchill Livingstone, New York, 1992, pp721-747

61）Yonekawa Y, Ogata N, Kaku Y, Taub E, Imhof HG：Moyamoya disease in Europe, past and present status. Clin Neurol Neurosurg 99 (suppl 2)：S58-S60, 1997

62）Yonekawa Y, Imhof HG, Taub E, Curcic M, Kaku Y, Roth P, Wieser HG, Groscurth P：Supracerebellar transtentorial approach posterior temporomedial structures. J Neurosurg 94：339-345, 2001

63）Yonekawa Y, Fandino J, Hug M, et al：Moyamoya angiopathy in Europe. In Cho BK, Tominaga T (eds)：Moyamoya disease Update. Springer, Tokyo, 2010, pp337-344

64）Yonekawa Y, Takagi Y, Khan N：Adult Moyamoya disease．Chapter 356. In Winn HR（ed）：Youmans：Neurological Surgery. Elsevier, New York, 2011, pp3680-3697