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1 Continuing Education Column Update of Moyamoya Disease Ji Yeoun Lee, MDSeungKi Kim, MD Division of Pediatric Neurosurgery, Seoul National University College of Medicine nsthomas@snu.ac.kr J Korean Med ssoc 2007; 50(12): bstract Moyamoya disease is characterized by bilateral stenosis or occlusion of distal internal carotid artery (IC) bifurcation including its proximal branches and abnormal vascular network (moyamoya vessel, MMV) in the vicinity of the arterial occlusions. It is the most common pediatric cerebrovascular disease in Eastern sia, particularly in Korea and Japan. The etiology is still unknown, but much about the pathology from autopsies, factors involved in its pathogenesis, and its genetics have been studied and reported. It may cause ischemic attacks or cerebral infarctions in children and cerebral hemorrhage in adults. ecause of its aggressive clinical course in very young children, the need for early detection and treatment has been recognized. Magnetic resonance imaging (MRI)/MR angiography (MR), cerebral hemodynamic studies, and cerebral angiography are used for the diagnosis. The treatment basically focuses on prevention of further ischemia and infarction through revascularization. Technically, direct and indirect bypass methods are used. The treatment strategy needs to be individualized in each patient. Outcomes of revascularization procedures are excellent in preventing transient ischemic attacks (TIs) in most patients. Keywords : Children; Moyamoya disease; Outcome; Revascularization 1109

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internal carotid and middle cerebral arteries () and huge

curvilinear flow voids of the hypertrophied moyamoya

3 Moyamoya Disease C D Figure 1. n axial T2weighted MR image shows diminished flow voids in the internal carotid and middle cerebral arteries () and huge cortical infarction in the right hemisphere and left frontal lobe (). n axial T1weighted MR image demonstrates punctuate and curvilinear flow voids of the hypertrophied moyamoya collateral in the basal ganglia (C). n axial T1weighted MR image with gadolinium enhancement reveals prominent leptomeningeal enhancement in the right hemisphere (D). 1111

4 Lee JY Kim SK Figure 2. Rest () and acetazolamide () SPECT images show decreased perfusion with disturbed vasoreactivity to the acetazolamide injection in the right hemisphere. Figure 3. perfusion MRI demonstrates an increased rcv () and delayed TTP () in the right hemisphere. 1112

stenotic change in the terminal portion of the internal

and stenotic change in the proximal anterior cerebral artery.

carotid artery and the middle cerebral artery, and total

5 Moyamoya Disease C D Figure 4. right carotid angiography (: P view, : lateral view) shows stenotic change in the terminal portion of the internal carotid artery, total occlusion in the middle cerebral artery and stenotic change in the proximal anterior cerebral artery. left carotid angiography (C: P view, D: lateral view) reveals stenotic change in the terminal portion of the internal carotid artery and the middle cerebral artery, and total occlusion in the anterior cerebral artery. 1113

Lee JY Kim SK STfrontal branch Scalp incision Figure 5. Operative illustrations of EDS operation. ) The skin incision was made along the course of the right ST.

Scalp incision STparietal branch Coronal Suture Dura Left galeo (periosteal) flap Superior sagittal sinus STparietal branch Right galeo (periosteal) flap Figure 6.

6 Lee JY Kim SK STfrontal branch Scalp incision Figure 5. Operative illustrations of EDS operation. ) The skin incision was made along the course of the right ST. ) The STgaleal flap laid on the exposed cortex was sutured to the incised edge of the dura mater after dissection of the arachnoid membrane (Modified from (33) with the permission from Ilchokak). Scalp incision STparietal branch Coronal Suture Dura Left galeo (periosteal) flap Superior sagittal sinus STparietal branch Right galeo (periosteal) flap Figure 6. Operative illustrations of bifrontal EG(P)S. ) Sshaped scalp incision was made 2 cm anterior to the coronal suture. ) The prepared galeo (periosteal) flap was inserted into the cerebral cortex and sutured to the dura (Modified from (35) with the permission from Ilchokak). 1114

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8 Lee JY Kim SK 11. Suzuki J, Takaku. Cerebrovascular "moyamoya" disease. Disease showing abnormal netlike vessels in base of brain. rch Neurol 1969; 20: Natori Y, Ikezaki K, Matsushima T, Fukui M. 'ngiographic moyamoya' its definition, classification, and therapy. Clin Neurol Neurosurg 1997; 99(S2): S Ikezaki K, Han DH, Kawano T, Kinukawa N, Fukui M. clinical comparison of definite moyamoya disease between South Korea and Japan. Stroke 1997; 28: Suzuki J, Kodama N. Moyamoya disease a review. Stroke 1983; 14: Nakashima H, Meguro T, Kawada S, Hirotsune N, Ohmoto T. Long--term results of surgically treated moyamoya disease. Clin Neurol Neurosurg 1997; 99(S2): S Ikezaki K. Clinical manifestations: epidemiology, symptoms and signs, laboratory findings. In: Ikezaki K, Loftus C, eds. Moyamoya Disease. Rolling Meadows: merican ssociation of Neurological Surgeons 2001; Wakai K, Tamakoshi, Ikezaki K, Fukui M, Kawamura T, oki R, Kojima M, Lin Y, Ohno Y. Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg 1997; 99(S2): S Kim SK, Wang KC, Kim DG, Paek SH, Chung HT, Han MH, hn Y, Cho K. Clinical feature and outcome of pediatric cerebrovascular disease: a neurosurgical series. Childs Nerv Syst 2000; 16: Fukui M, Kono S, Sueishi K, Ikezaki K. Moyamoya disease. Neuropathology 2000; 20(S): S Malek M, Connors S, Robertson RL, Folkman J, Scott RM. Elevation of cerebrospinal fluid levels of basic fibroblast growth factor in moyamoya and central nervous system disorders. Pediatr Neurosurg 1997; 27: Hojo M, Hoshimaru M, Miyamoto S, Taki W, Nagata I, sahi M, Matsuura N, Ishizaki R, Kikuchi H, Hashimoto N. Role of transforming growth factorbeta1 in the pathogenesis of moyamoya disease. J Neurosurg 1998; 89: Kim SK, Yoo JI, Cho K, Hong SJ, Kim YK, Moon J, Kim JH, Chung YN, Wang KC. Elevation of CRPI in the cerebrospinal fluid of patients with Moyamoya disease. Stroke 2003; 34: Yamada H, Deguchi K, Tanigawara T, Takenaka K, Nishimura Y, Shinoda J, Hattori T, ndoh T, Sakai N. The relationship between moyamoya disease and bacterial infection. Clin Neurol Neurosurg 1997; 99(S2): S Ikezaki K, Kono S, Fukui M. Etiology of moyamoya disease: pathology, pathophysiology, and genetics. In: Ikezaki K, Loftus C, eds. Moyamoya Disease. Rolling Meadows: merican ssociation of Neurological Surgeons, 2001: Kang HS, Kim SK, Cho K, Kim YY, Hwang YS, Wang KC. Single nucleotide polymorphisms of tissue inhibitor of metalloproteinase genes in familial moyamoya disease. Neurosurgery 2006; 58: Kim SK, Seol HJ, Cho K, Hwang YS, Lee DS, Wang KC. Moyamoya disease among young patients: its aggressive clinical course and the role of active surgical treatment. Neurosurgery 2004; 54: ; discussion Ogawa, Nakamura N, Yoshimoto T, Suzuki J. Cerebral blood flow in moyamoya disease. Part 2: utoregulation and CO2 response. cta Neurochir (Wien) 1990;105: Seol HJ, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho K. Headache in pediatric moyamoya disease: review of 204 consecutive cases. J Neurosurg 2005; 103: Ikezaki K, Fukui M, Inamura T, Kinukawa N, Wakai K, Ono Y. The current status of the treatment for hemorrhagic type moyamoya disease based on a 1995 nationwide survey in Japan. Clin Neurol Neurosurg 1997; 99(S2): S Saeki N, Nakazaki S, Kubota M, Yamaura, Hoshi S, Sunada S, Sunami K. Hemorrhagic type moyamoya disease. Clin Neurol Neurosurg 1997; 99(S2): S Iwama T, Morimoto M, Hashimoto N, Goto Y, Todaka T, Sawada M. Mechanism of intracranial rebleeding in moyamoya disease. Clin Neurol Neurosurg 1997; 99(S2): S Houkin K, Yoshimoto T, Kuroda S, Ishikawa T, Takahashi, be H. ngiographic analysis of moyamoya diseasehow does moyamoya disease progress? Neurol Med Chir (Tokyo). 1996; 36: ; discussion Kuroda S, Kamiyama H, Isobe M, Houkin K, be H, Mitsumori K. Cerebral hemodynamics and "rebuildup" phenomenon on electroencephalogram in children with moyamoya disease. Childs Nerv Syst 1995; 11: Ikezaki K, Matsushima T, Kuwabara Y, Suzuki SO, Nomura T, Fukui M. Cerebral circulation and oxygen metabolism in childhood moyamoya disease: a perioperative positron emission tomography study. J Neurosurg 1994; 81: Kim SK, Wang KC, Oh CW, Kim IO, Lee DS, Song IC, Cho K. Evaluation of cerebral hemodynamics with perfusion MRI in childhood moyamoya disease. Pediatr Neurosurg 2003; 38: Kuwabara Y, Ichiya Y, Sasaki M, Yoshida T, Masuda K, Matsushima T, Fukui M. Response to hypercapnia in moyamoya disease. Cerebrovascular response to hypercapnia in pediatric and adult patients with moyamoya disease. Stroke 1997; 28: Seol HJ, Wang KC, Kim SK, Lee CS, Lee DS, Kim IO, Cho K. Unilateral (probable) moyamoya disease: longterm followup of seven cases. Childs Nerv Syst 2006; 22: Okada Y, Shima T, Matsumura S, Nishida M, Yamada T, Okita 1116

9 Moyamoya Disease S. [Pathophysiological studies in moyamoya disease by rcf and cortical artery pressure measurements in comparison to those in IC or MC occlusion]. No To Shinkei 1988; 40: Matsushima Y, Inaba Y. Moyamoya disease in children and its surgical treatment. Introduction of a new surgical procedure and its followup angiograms. Childs rain 1984; 11: Kim SK, Wang KC, Kim IO, Lee DS, Cho K. Combined encephaloduroarteriosynangiosis and bifrontal encephalogaleo (periosteal)synangiosis in pediatric moyamoya disease. Neurosurgery 2002; 50: Kim CY, Wang KC, Kim SK, Chung YN, Kim HS, Cho K. Encephaloduroarteriosynangiosis with bifrontal encephalogaleo (periosteal) synangiosis in the pediatric moyamoya disease: the surgical technique and its outcomes. Childs Nerv Syst 2003; 19: Chung YN, Wang KC, Cho K. Moyamoya disease, Encephaloduroarteriosynangiosis. In: Kim DG,ed. Practical Points in Neurosurgery. Seoul: Ilchokak, 2007: Kim SK, Wang KC, Cho K. Moyamoya disease, ifrontal Encephalogaleo (periosteal) synangiosis. In: Kim DG,ed. Practical Points in Neurosurgery. Seoul: Ilchokak, 2007: Choi IJ, Hong SH, Cho K, Wang KC, Kim SK. Encephalo duroarteriosynangiosis (EDS) using occipital artery in children with moyamoya disease. Journal of Korean Neurosurgical Society 2005; 38: SainteRose C, Oliveira R, Puget S, enidani L, oddaert N, Thorne J, Wray, Zerah M, ourgeois M. Multiple bur hole surgery for the treatment of moyamoya disease in children. J Neurosurg 2006; 105: Karasawa J, Touho H, Ohnishi H, Miyamoto S, Kikuchi H. Longterm followup study after extracranialintracranial bypass surgery for anterior circulation ischemia in childhood moyamoya disease. J Neurosurg 1992; 77:

10 Lee JY Kim SK Peer Reviewer Commentary 1118

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untitled Review Article Ewha Med J 2013;36(1):9-17 pissn 2234-3180 / eissn 2234-2591 http://dx.doi.org/10.12771/emj.2013.36.1.9 모야모야병의진단과치료 서의교 이화여자대학교의학전문대학원신경외과학교실 Diagnosis and Treatment of Moyamoya Disease