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1 Review Article Ewha Med J 2013;36(1):9-17 pissn / eissn 모야모야병의진단과치료 서의교 이화여자대학교의학전문대학원신경외과학교실 Diagnosis and Treatment of Moyamoya Disease Eui Kyo Seo Department of Neurosurgery, Ewha Womans University School of Medicine, Seoul, Korea Moyamoya disease is a cerebrovascular disease of unknown etiology, which is characterized by bilateral stenosis or occlusion at terminal portion of internal carotid artery and at proximal portion of anterior cerebral artery and /or middle cerebral artery and abnormal vascular network in the vicinity of the arterial occlusions. It occurs frequently in Asian countries, particularly in Korea and Japan, but is rare in Western countries. To establish the etiology of moyamoya disease, much about the pathology from autopsies, factors involved in its pathogenesis, and its genetics have been studied. It may occur at any age from childhood to adulthood and in general, initial manifestation is cerebral ischemic symptoms in children and intracranial hemorrhage symptoms in adults. Because it progress and cause recurrent stroke, early diagnosis and proper management has been recognized. Cerebral angiography is essential for definitive diagnosis and treatment plan. Magnetic resonance imaging/magnetic resonance angiography is useful for diagnosis and follow-up tools after revascularization. Evaluation of the cerebral hemodynamics by single photon emission computed tomography and positron emission tomography is useful for diagnosis and assessment of the severity of cerebral ischemia in moyamoya patients. Surgical revascularization is effective for moyamoya disease manifesting as ischemic symptoms, to prevent further ischemia and infarction. In hemorrhagic type moyamoya disease, revascularization can be considered. Direct bypass, indirect synangiosis and combined methods are used. Outcomes of revascularization are excellent in preventing transient ischemic attacks in most patients. (Ewha Med J 2013;36(1):9-17) Key Words: Cerebral hemorrhage; Cerebral ischemia; Moyamoya disease; Revascularization 서론 모야모야병 (moyamoya disease) 은특별한원인없이내경동맥의원위부나그분지인중대뇌동맥이나전대뇌동맥의근위부에협착이생기고점차진행하여폐색이일어나고모야모야혈관이라고하는비정상적인 Received: January 26, 2013, Accepted: March 2, 2013 Corresponding author: Eui Kyo Seo, Department of Neurosurgery, Ewha Womans University School of Medicine, 1071 Anyangcheon-ro, Yangcheon-gu, Seoul , Korea Tel: , Fax: drekseo@ewha.ac.kr 이상혈관이뇌기저부에관찰되는만성진행성뇌혈관질환이다. 서양보다는동양특히동아시아에서많이발생하는것으로알려져있다. 1957년일본에서처음으로증례가보고되었으며 1960년대병이름으로 모야모야 라는용어가사용되었으며체계적으로뇌혈관조영술의소견과임상증상에대해기술되기시작하였다 [1]. 모야모야 는일본말로뇌혈관조영술에서뇌기저부의비정상적인이상혈관이마치모락모락피어오르는담배연기 (hazy cloud like puff of cigarette smoke) 처럼보인다고해서붙여진이름이다. 뇌동맥의협착과폐색이양측으로진행하면모야모야병으로확진하 9

2 Ewha Med J Vol. 36, No. 1, 2013 며, 일측성으로한쪽에만생기면모야모야병으로추정한다. 뇌혈관폐색과관련하여비슷한용어가있는데, 뇌동맥죽상경화증, 혈관염, 자가면역질환등혈관폐색을일으킨원인이있을때는모야모야증후군이라고한다 [2,3]. 어린이와어른에게서모두발병하는것으로알려져있으며어린이는주로반복적인일과성뇌허혈발작 (transient ischemic attack) 과뇌경색의형태로나타나고어른에게서는뇌출혈이흔하다 [4,5]. 모야모야병은아직그원인이알려져있지않아이질환을막을수있는근본적인치료법은없다. 뇌동맥폐색에대한수술적치료인혈관문합술의효과는여러보고를통해입증되어있다 [6,7]. 이에본연구에서는지금까지의모야모야병에대한연구들을검토해보고일반적으로받아들여지는역학, 병리소견, 임상증상, 진단방법, 치료및예후에대해알아보고자한다. 본론 1. 역학병리모야모야병발병의지역적차이는매우커서한국, 일본등의극동아시아에서호발하고유럽과미대륙의사람들에는드물다. 한연구에서는세계에서환자의분포를조사하였는데일본을제외하고 1,063명의환자중아시아 625명 ( 한국 289명, 중국 245명 ), 유럽 201명, 남북아메리카 176명으로아시아에서흔하게발병하는것으로알려졌다 [8]. 일본에서의대규모역학연구에따르면인구 10만명당 3.16명이이병을가지고있으며 0.35명이발병한다 [9,10]. 남녀의비율은 1: 로여자에게서많이발병한다 [9]. 환자의 10% 에서가족력이있으며 [9,10], 일반인에비해환자의형제는 30배정도환자의자녀는 40배정도발병의위험성이높다 [9]. 증상이발생하는연령은 10세이전에가장잘생기고 30대에두번째로잘발병하는것으로알려져있다 [9-11]. 모야모야병으로인한출혈은 50대에가장잘생기는것으로알려져있다 [12]. 최근건강검진등으로뇌자기공명영상 (magnetic resonance imaging, MRI) 을많이시행함으로인해증상이없는모야모야병이진단되고그비율이점차증가하고있으며최근역학조사에서는증상없는모야모야병의유병률이인구 10만명당 10.5명으로조사되었다 [13]. 부검을통하여모야모야병의주된소견이내경동맥의말단부의협착과폐색, 모야모야혈관의형성, 측부순환의형성이다. 조직학적소견으로내경동맥내 막의증식, 비후된내막의지방축적, 내탄력층의물결모양의변형, 윌리스환주위의작은혈관채널, 연막에작은혈관채널이특징적이다 [14]. 모야모야병의원인과관련하여여러인자에대해서연구가진행되고있는데 basic fibroblast growth factor, transforming growth factor-1, platelet derived growth factor, interleukin-1, -2, cellular retinoic acidbinding protein-i 등의인자와수용체가모야모야병환자의표재측두동맥 (superficial temporal artery), 혈액혹은뇌척수액에서과발현됨이보고되었다 [15,16]. 가족성모야모야병에서유전자의위치가 3p24-p26, 8q25, 6q25, 17q25 등여러곳으로알려졌으며, 3세대이상에걸쳐상염색체우성으로유전된다 [17]. 일측성의모야모야병이양측으로진행하는환자의유전자검사를통해가족성모야모야병과비교하여이병을진행하게하는유전자의위치를찾는노력이진행되고있다. 2. 임상증상및징후처음증상에따라출혈형, 간질형, 뇌경색형, 일과성허혈형, 빈번한일과성허혈형, 두통형, 무증상형, 기타로분류하는데일과성허혈형이가장많고출혈형, 뇌경색형, 빈번한일과성허혈형, 두통형의순서로많다 [10]. 증상은연령과병의진행형태에따라아주다양하게나타난다. 뇌허혈에의한증상은어린이에서많이나타나는데어린이환자의약 70% 를차지한다. 특히 3세이하의어린이에서는병의진행이빠르며뇌경색이많이발생하는데뇌출혈은거의생기지않는다 [10]. 이러한뇌허혈성증상은과호흡후에많이유발되는데심한운동을하거나심하게울때, 하모니카를불거나뜨거운음식을불며식히는과정에서자주발생하게된다. 뇌허혈증상으로는운동마비, 의식저하, 감각이상, 간질, 두통, 언어장애, 감각이상등이흔하고이런증상은갑자기생기며주로같은쪽으로반복되고진행하기도한다. 병이진행하여뇌허혈증상이반복되어뇌위축이유발되고뇌경색으로악화되어심각한지능장애를초래한다. 어른에게서는주로뇌출혈이생기는데여자에게서출혈이잘생기는것으로알려져있으며의식저하, 두통, 운동마비, 언어장애가나타나며뇌실내출혈이많고사망률이높으므로조심해야한다. 두통은어린이에게서많이나타나고편두통처럼심한경우에서부터묵직한두통까지다양하게나타나며 10 THE EWHA MEDICAL JOURNAL

Seo EK: Diagnosis and Treatment of Moyamoya Disease 아직그발생기전은정확히알려져있지않다 [18]. 3. 진단모야모야병의진단에뇌혈관조영술은필수적이며치료방침을결정하고수술결과를평가하는표준방법이다.

최근에는 MRI/magnetic resonance angiography (MRA) 의정확도가높아짐에따라모야모야병의진단에이용되고있다. 정확한영상을얻기위해 1.

모야모야병의뇌허혈정도를측정하고치료방침과예후를결정하기위해서는양전자방출단층촬영 (positron emission tomography, PET) 이나단광자방출단층촬영 (single photon emission computed tomography, SPECT) 을이용한뇌혈류및뇌대사상태를측정해야한다.

PET에서는국소뇌혈류 (regional cerebral blood flow) 와국소산소대사율 (regional cerebral metabolic rate of oxygen) 이감소하고반면에국소뇌혈류 Fig. 1.

3 Seo EK: Diagnosis and Treatment of Moyamoya Disease 아직그발생기전은정확히알려져있지않다 [18]. 3. 진단모야모야병의진단에뇌혈관조영술은필수적이며치료방침을결정하고수술결과를평가하는표준방법이다. 내경동맥말단부나전대뇌동맥이나중대뇌동맥의기시부의폐색이다양하게나타나고기저부에비정상적인작은혈관들 ( 모야모야혈관 ) 이보인다 (Fig. 1). 모야모야병의진행여부를알아보는데도뇌혈관조영술이가장유용하다 (Fig. 2). 최근에는 MRI/magnetic resonance angiography (MRA) 의정확도가높아짐에따라모야모야병의진단에이용되고있다. 정확한영상을얻기위해 1.5 T 이상의기기로검사할것을권장하고내경동맥의말단부나전대뇌동맥이나중대뇌동맥의기시부의협착이나폐색이보이며기저핵주위로비정상적인혈관채널즉모야모야혈관이보이며이런소견이양측으로보일때확정적모야모야병이라고진단 한다. 기저핵주위에 2개이상의 signal void가보이면비정상적혈관채널이라고판단할수있다 (Fig. 3) [19]. 모야모야병의뇌허혈정도를측정하고치료방침과예후를결정하기위해서는양전자방출단층촬영 (positron emission tomography, PET) 이나단광자방출단층촬영 (single photon emission computed tomography, SPECT) 을이용한뇌혈류및뇌대사상태를측정해야한다. 뇌허혈상태에서뇌산소대사를유지하기위한보상작용이일어나는데, 모야모야병에서뇌동맥의폐색으로인한뇌관류압 (cerebral perfusion pressure) 이심하게감소되어뇌혈관의확장반응만으로뇌혈류 (cerebral blood flow, CBF) 를유지할수없게되어보상적으로뇌혈류량을증가시키게되고산소추출율은증가시킨다 [20,21]. PET에서는국소뇌혈류 (regional cerebral blood flow) 와국소산소대사율 (regional cerebral metabolic rate of oxygen) 이감소하고반면에국소뇌혈류 Fig. 1. A left carotid angiography (A, anteroposterior view; B, lateral view) shows severe stenosis in terminal portion of internal carotid artery and proximal middle cerebral artery and occlusion of proximal portion of anterior cerebral artery and demonstrates basal collateral vessels. A right carotid angiography (C, anteroposterior view; D, lateral view) demonstrates stenosis of proximal middle cerebral artery and occlusion in proximal portion of anterior cerebral artery. THE EWHA MEDICAL JOURNAL 11

Initial angiography (A) shows mild stenosis in terminal portion of internal carotid artery and well visualized middle cerebral artery.

head) from posterior cerebral artery. Posterior cerebral artery is occluded and collateral vessel is disappeared after 6 months later (C).

$량(regional cerebral blood volume)과 국소 산소추출률 (regional oxygen extraction fraction)은 증가된다 [20,21].$ SPECT는 국소 뇌혈류를 알 수 있고, acetazolamide 정맥 주사 전후를 비교하면 혈류 예비능(vascular reserve)을 측정할 수 있다(Fig. 4) [22].

SPECT는 국소 뇌혈류를 알 수 있고, acetazolamide 정맥 주사 전후를 비교하면 혈류 예비능(vascular reserve)을 측정할 수 있다(Fig. 4) [22].

4 Ewha Med J Vol. 36, No. 1, 2013 Fig. 2. Initial angiography (A) shows mild stenosis in terminal portion of internal carotid artery and well visualized middle cerebral artery. After 2 years, angiography (B) demonstrate that middle cerebral artery is not visualized and basal there are collateral vessels (arrow head) from posterior cerebral artery. Posterior cerebral artery is occluded and collateral vessel is disappeared after 6 months later (C). Fig. 3. A T2 weighted axial magnetic resonance image (A) shows cerebral infarction in right temporo-occipital areas. An axial T1 weighted image (B) demonstrates multiple signal voids of the hypertrophied moyamoya collateral in the basal ganglia. 량(regional cerebral blood volume)과 국소 산소추출률 (regional oxygen extraction fraction)은 증가된다 [20,21]. SPECT는 국소 뇌혈류를 알 수 있고, acetazolamide 정맥 주사 전후를 비교하면 혈류 예비능(vascular reserve)을 측정할 수 있다(Fig. 4) [22]. 수술 전후의 결 과를 비교하여 수술의 효과를 알아보는데도 유용하게 사용할 수 있다. 경두개도플러(transcranial Doppler), perfusion MRI, perfusion CT도 뇌혈류상태를 평가하 는데 이용되고 있다. 모야모야병의 볼 수 있는 특징적인 뇌파는 과호흡 시에 규칙적인 고진폭의 서파가 출현하고(build-up), 12 THE EWHA MEDICAL JOURNAL 과호흡이 끝난 수분 후에 불규칙해진 고진폭의 서파 가 다시 나타나는(rebuild-up현상) 것으로 어린이에게 서 잘 나타난다. 이 rebuild-up 현상은 과호흡에 의해 유발된 과이산화탄소혈증에 의해서 대뇌피질의 뇌혈 류가 회복이 늦어져 생기는 것으로 추측된다[23]. 4. 치료 Chiu 등[24]의 연구에 의하면 치료하지 않은 모야모 야병의 장기추적결과 매년 10.3%의 뇌졸중 발생률을 보였는데 뇌졸중은 진단 첫해에 18%, 그리고 그 이후 는 매년 5%의 발생률을 보였다. 뇌혈관의 폐색정도도

진행하고그진행정도에따라출혈이나뇌경색이더잘생기며재출혈의위험도높아진다 [5,25,26]. 모야모야병이진행함에따라심각한신경학적이상증상을초래할수있어서치료를고려하는데치료의목표는반복되는일과성뇌허혈발작을막고, 병이진행되지않도록하여뇌경색을예방하고뇌출혈을막는것이다.

5 Seo EK: Diagnosis and Treatment of Moyamoya Disease Fig. 4. Basal single photon emission computed tomography (SPECT) (A) and acetazolamide SPECT (B) show decreased blood flow and reserve capacity in left frontal lobe. 진행하고그진행정도에따라출혈이나뇌경색이더잘생기며재출혈의위험도높아진다 [5,25,26]. 모야모야병이진행함에따라심각한신경학적이상증상을초래할수있어서치료를고려하는데치료의목표는반복되는일과성뇌허혈발작을막고, 병이진행되지않도록하여뇌경색을예방하고뇌출혈을막는것이다. 뇌혈류량과혈류예비능을호전시키기위해서는보존적인치료보다재관류술인수술적치료가더효과적인것으로알려져있다 [20,27]. 모야모야병은환자상태에따라수술시기와수술방법및그적응증이달라진다. 뇌허혈발작환자에서광범위한뇌경색이없고회복이가능한뇌허혈에의한증상만있으며 SPECT, PET 등의뇌혈류검사에서뇌혈류량이감소되어있고 acetazolamide 정맥주사검사로혈류예비능이저하되어있을때수술로좋은효과를볼수있다. 증상이없는모야모야병이라도진행의가능성이높은어린이에게서뇌혈류검사결과에따라수술하는경우가있으나어른에게서는권유되지않는다 [28]. 한쪽뇌혈관이막힌추정모야모야병의경우, 2세이하의어린이에서는양측성으로진행하는경우가많으나나머지환자군에서는이러한진행을보이지않는경우도있어증상이있는반구에대해서만수술을해주고나머지반구에대해서는집중적추적관찰을하는것이권유된다 [29]. 3세이하의어린이의경우병의진행이더빨라발견당시뇌경색이있는경우가많고수술을기다리는기간동안에도추가적인뇌경색이발생하는경우가 39% 까지나타나조기에수술을해 주는것이필요하다 [30,31]. 성인모야모야병에서출혈성뇌졸중의발생은지속적인혈역학적과부하에노출된기저모야모야혈관의파열에의한것으로알려져있다. 그래서주로기저핵, 시상혹은뇌실주변의백질에서출혈이시작되고, 뇌실내출혈로확장되는경우가빈번하다 [32,33]. 혈관촬영에서측부순환의혈관이나모야모야혈관에서미세동맥류가발견되는경우도있다. 모야모야병의측부순환에중요한역할을하는후순환계뇌동맥에서지속적인혈역학적스트레스에의한낭성뇌동맥류가잘발생하고이로인한뇌지주막하출혈의발생도드물지않다 [34]. 출혈성모야모야병은특히예후가좋지않아재출혈률이연간 7.09% 로보고되고있다 [26]. 장기적으로재출혈을예방할수있는치료가절실히필요하고직간접혈관재건술을통한측부순환의발달이궁극적으로혈류역학적과부하를경감시켜재출혈을예방할수있을것이라는기대때문에여러기관에서혈관재건술을시도하였지만그효과에대해서는대단히의견이분분하다. 다양한종류의혈관재건술을시행하였지만재출혈예방에유의한실효를거두지못했다는보고가있는가하면 [7,35], 직간접동시재건술을시행한군에서뚜렷하게재출혈이감소되는경향이있고혈관촬영소견에서말초혈관의미세동맥류가사라지거나혈류역학적스트레스안정화되는현상을관찰하였으므로잠재적으로유효할가능성을시사한연구들이발표되고있기도하다 [25,36]. 출혈성모야모야병환자의효과적인치료법에대한대규모연구결 THE EWHA MEDICAL JOURNAL 13

6 Ewha Med J Vol. 36, No. 1, 2013 과는아직없으나, 2001년일본전국 23개기관이참여한다기관무작위공동연구 (Japanese adult moyamoya trial) 가시작되어곧발표될결과에관심이크다. 직접재관류 (direct revascularization) 수술은표재측두동맥과중대뇌동맥의피질분지사이의문합을통해이루어지는데, 혈액관류가적은뇌부분에직접재관류를시켜줄수있고수술직후부터혈류량이증가된다는장점이있다. 반면에혈관이작으면수술이어렵고국소뇌혈류를막아서뇌경색이유발되는경우가있으며재관류가이미생성된측부순환을방해할가능성도있어서주의해야한다. 성인의경우신생혈관형성이잘되지않아간접재관류수술의효과가제한적이어서직접재관류술이효과가좋은것으로알려져있다 [37]. 간접재관류 (indirect revascularization) 수술은직접재관류술에비해덜침습적이며수술술기가쉬운장점이있으나신생혈관생성에시간이걸리며목표로한대뇌피질에재관류가충분하지못할수있는단점이있다. 간접재관류술에는여러가지방법이있는데뇌경막, 측두근육, 중경막동맥 (middle meningeal artery), 모상건막 (galea aponeurotica), 표재측두동맥과같은혈관분포가풍부한표재조직을뇌표면에접촉하게하여신생혈관이형성되도록하여, 두개강바깥의외경동맥분지와두개강안쪽의내경동맥의뇌피질분지와문합을이루어혈류가통하게하는것이다 [38]. 나이어린어린이의경우혈관이너무작아직접재관류술은술기상어려움이많아서간접재관류술을많이사용하고있다. 성인에서는직접재관류술을많이시행하는데서로의단점을보완하기위해혼합형재관류술을시행하기도한다. 중대뇌동맥의영역에 주로재관류술을시행하였는데인지기능에중요한역할을하는전대뇌동맥의영역에도혈류량을증가시키기위해전두엽에모상건막을삽입하는등다양한수술방법이행해지고있다. 모야모야병이진행하여후순환계 (posterior circulation) 까지파급되면후대뇌동맥영역에뇌혈류를증가시키기위해후두동맥을이용한 encephalodural arterial synangiosis나 multiple burr holes 수술등이시행된다 [39]. 수술과관련된합병증으로는뇌졸중, 뇌경막하수종및혈종, 과관류증후군, 창상감염등이있으며, 가장문제가되는것은수술중혹은수술후에발생되는뇌졸중이다 [31,40]. 수술후뇌졸중은 3.8% 에서 22.2% 까지보고되고있다 [31,40]. 이와같은합병증을예방하기위하여는수술중에정상이산화탄소분압, 정상혈압, 정상체온을반드시유지하고충분한수액공급과빈혈교정의필요성을강조하였다. 그외에도수술직후수술통증으로심하게울면서초래되는과호흡상태때문에도뇌허혈증상이나타날수있으므로수술직후통증관리에세심한배려가필요하다. 5. 예후, 추적검사영아에서뇌경색의유무가예후에가장중요한요인이다. 일측성인추정모야모야병이양측성으로진행하고증상이없던환자도시간이지남에따라약 65% 에서일과성뇌허혈발작이발생한다 [41]. 많은연구에서재관류술후두통도호전되며일과성허혈발작은많이줄어들거나없어지고뇌경색도아주감소하였으며뇌기능도호전되어지능도좋아지는것으로나타났다 [18,24,34]. Fig. 5. After revascularization, cerebral blood flow is increased in left frontal lobe. 14 THE EWHA MEDICAL JOURNAL

7 Seo EK: Diagnosis and Treatment of Moyamoya Disease 모야모야병에서뇌출혈로인한사망률은 6.8% 에서 20% 까지보고되고있으며 30 60% 가재출혈한다고알려져있다. 증상이없는모야모야병도점차진행하여뇌경색이 40% 에서발생한다고하며, 연간출혈의위험성은 3.2% 로보고되고있다 [42]. 따라서증상이없는모야모야병도뇌졸중의위험이있는환자로관리해야할것이다. 모야모야병의추적조사시기와방법에대해서는확실히정립이되지않았다. 직접재관류술을시행한경우수술후 6개월에뇌혈관조영술과 SPECT를시행하여수술에따른효과를확인해보는것이권장되고 (Fig. 5), 간접재관류술을시행한환자는수술후 6개월 2년에뇌혈관조영술과 SPECT를시행하는것이좋다 [43,44]. 증상이있으나치료를하지않은환자는매년 MRI/MRA로병이진행하는지알아보며증상이없는모야모야병환자는주기적인검사보다는필요하면그때그때 MRI/MRA 를시행하는것이좋다. 새로운증상이생기면반드시뇌혈관조영술과 PET, SPECT를시행하여치료방침을정해야한다 [44]. 결론 모야모야병의발병기전이아직정확하게규명되어있지않아서원인규명을통한예방, 조기진단, 진행방지및근원적치료는불가능한실정이고질환자체가비교적최근에알려져서대부분의연구가단기추적검사결과만포함하고있고질병의진행이만성적인경과를취하고다양한임상경과를보이기때문에장기적인임상경과나예후는알려져있지않다. 더많은연구를통해모야모야병의병인이밝혀지고치료법이개발될것을기대한다. 참고문헌 1. Suzuki J, Takaku A. Cerebrovascular "moyamoya" disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 1969;20: Natori Y, Ikezaki K, Matsushima T, Fukui M. 'Angiographic moyamoya' its definition, classification, and therapy. Clin Neurol Neurosurg 1997;99 Suppl 2:S168-S Czartoski T, Hallam D, Lacy JM, Chun MR, Becker K. Postinfectious vasculopathy with evolution to moyamoya syndrome. J Neurol Neurosurg Psychiatry 2005;76: Suzuki J, Kodama N. Moyamoya disease: a review. Stroke 1983;14: Kuroda S, Ishikawa T, Houkin K, Nanba R, Hokari M, Iwasaki Y. Incidence and clinical features of disease progression in adult moyamoya disease. Stroke 2005;36: Nakashima H, Meguro T, Kawada S, Hirotsune N, Ohmoto T. Long-term results of surgically treated moyamoya disease. Clin Neurol Neurosurg 1997;99 Suppl 2:S156-S Okada Y, Shima T, Nishida M, Yamane K, Yamada T, Yamanaka C. Effectiveness of superficial temporal artery-middle cerebral artery anastomosis in adult moyamoya disease: cerebral hemodynamics and clinical course in ischemic and hemorrhagic varieties. Stroke 1998;29: Goto Y, Yonekawa Y. Worldwide distribution of moyamoya disease. Neurol Med Chir (Tokyo) 1992;32: Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, et al. Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg 1997;99 Suppl 2:S1-S Ohki K, Hoshino H, Nogawa S, Yamaguchi K Databases evaluation by the Research Committee on Moyamoya Disease (Spontaneous occlusion of the circle of Willis). In: Ministry of Health, Labour and Welfare, editor. Research on spontaneous occlusion of the circle of Willis. Tokyo: Ministry of Health, Labour and Welfare; p Ikezaki K. Clinical manifestations: epidemiology, symptoms and signs, laboratory findings. In: American Association of Neurological Surgeons, editor. Moyamoya disease. Rolling Meadows (IL): American Association of Neurological Surgeons; p Yamaguchi K, Nogawa S, Fukuuchi Y. National survey on spontaneous occlusion of the circle of Willis (moyamoya disease). Shinkei Naika 2001;54: Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 2008;79: Fukui M, Kono S, Sueishi K, Ikezaki K. Moyamoya disease. Neuropathology 2000;20 Suppl:S61-S Malek AM, 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, Asahi M, et al. Role of transforming growth factor-beta1 in the pathogenesis of moyamoya disease. J Neurosurg 1998;89: Mineharu Y, Takenaka K, Yamakawa H, Inoue K, Ikeda H, Kikuta KI, et al. Inheritance pattern of familial moyamoya disease: autosomal dominant mode and genomic THE EWHA MEDICAL JOURNAL 15

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9 Seo EK: Diagnosis and Treatment of Moyamoya Disease 1996;38: Nagata S, Matsushima T, Morioka T, Matsukado K, Mihara F, Sasaki T, et al. Unilaterally symptomatic moyamoya disease in children: long-term follow-up of 20 patients. Neurosurgery 2006;59: Kuroda S, Hashimoto N, Yoshimoto T, Iwasaki Y; Research Committee on Moyamoya Disease in Japan. Radiological findings, clinical course, and outcome in asymptomatic moyamoya disease: results of multicenter survey in Japan. Stroke 2007;38: Guzman R, Lee M, Achrol A, Bell-Stephens T, Kelly M, Do HM, et al. Clinical outcome after 450 revascularization procedures for moyamoya disease. Clinical article. J Neurosurg 2009;111: Andaluz N, Choutka O, Zuccarello M. Trends in the management of adult moyamoya disease in the United States: results of a nationwide survey. World Neurosurg 2010;73: THE EWHA MEDICAL JOURNAL 17

의학강좌_이지연

의학강좌_이지연 Continuing Education Column Update of Moyamoya Disease Ji Yeoun Lee, MDSeungKi Kim, MD Division of Pediatric Neurosurgery, Seoul National University College of Medicine Email : nsthomas@snu.ac.kr J Korean