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Kiyohiro Houkin, Hiroshi Abe, Tetsuyuki Yoshimoto and Akihiro Takahashi

T ypical moyamoya disease is a bilateral stenoocclusive process of the carotid artery fork. 9, 14, 15, 17, 18 The official criteria established by the Japanese Ministry of Health and Welfare for the diagnosis of moyamoya disease require bilateral stenoocclusive changes verified by conventional angiography, especially in adult patients. 15 Whether “unilateral” moyamoya disease, confirmed by typical angiographic evidence of moyamoya disease unilaterally and normal or equivocal findings contralaterally, is an early form of the typical (bilateral) moyamoya

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Gordon M. Burke, Allan M. Burke, Arun K. Sherma, Michael C. Hurley, H. Hunt Batjer and Bernard R. Bendok

M oyamoya disease is a chronic, occlusive cerebrovascular disease involving bilateral stenosis or occlusion of the terminal portion of the ICAs and/or the proximal portions of the ACAs and MCAs. 60 Moyamoya disease is also characterized by irregular perforating vascular networks, called moyamoya vessels, near the occluded or stenotic regions corresponding to the lenticulostriate and thalamoperforate arteries. It is this outgrowth of small vessels that produces the radiological image of a hazy “puff of smoke” giving the disease its name, “moyamoya” in

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Namio Kodama and Jiro Suzuki

T here have been few reported cases of moyamoya disease, 3–5, 7–10 and fewer still associated with aneurysms. 2, 5, 6 Up to now, we have experienced five such cases; in three patients the aneurysms arose from the posterior choroidal artery, and in two from the basilar artery. Based on these five cases, we discuss the formation process of these aneurysms and their symptomatic occurrence in moyamoya disease. Case Reports Case 1 This 16-year-old girl suddenly commenced vomiting on December 24, 1968, followed by loss of consciousness. On admission, the

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Does moyamoya disease cause subarachnoid hemorrhage?

Review of 54 cases with intracranial hemorrhage confirmed by computerized tomography

Nobuhiko Aoki and Hiroshi Mizutani

T he clinical presentation of moyamoya disease usually includes repeated transient ischemic attacks in children and intracranial hemorrhage in adults, particularly subarachnoid hemorrhage (SAH). 8, 14 However, in the majority of cases of moyamoya disease producing intracranial hemorrhage, computerized tomography (CT) has shown that the bleeding is intracerebral or intraventricular; therefore, whether moyamoya disease does cause primary hemorrhage into the subarachnoid space has become doubtful. In this paper, we discuss the site of hemorrhage and the

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Hiroshi Yamada, Shigetoshi Nakamura and Naoki Kageyama

O nce considered to be a specifically Japanese entity, 6 moyamoya disease or syndrome has been described in patients of other races. 1, 5, 10, 12, 14, 15 Two types of moyamoya syndrome have been differentiated; the infantile form, characterized clinically by recurring transient hemiparetic attacks, both uni- and contralateral, and the adult form, usually made manifest by subarachnoid hemorrhage. 7 In 1975, Kudo 7 reviewed the moyamoya syndrome in Japan. He found seven instances of familial occurrence among 363 cases of moyamoya disease. Kitahara, et al

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Mamoru Tomida, Masaaki Muraki and Kenji Yamasaki

M oyamoya disease—spontaneous occlusion of the circle of Willis—is progressive. It is generally believed that in children moyamoya disease advances in angiographically visible stages as classified by Suzuki, 12 whereas in adults the disease rarely progresses in stages. 2, 12 Whether an angiographically documented progression of changes from Suzuki's early stage occurs in adult as well as in pediatric cases is unknown. In this article we report an adult case of definite moyamoya disease with drastic angiographically documented changes progressing from Suzuki

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Ali A. Baaj, Siviero Agazzi, Zafar A. Sayed, Maria Toledo, Robert F. Spetzler and Harry van Loveren

blood supply as well as promote neoangiogenesis. Results from surgical intervention have been mixed. Overall, however, the response to surgical treatment appears to be favorable, particularly in decreasing recurrent ischemic events. 3 , 14 This article reviews the major surgical options available for the treatment of MMD. TABLE 1: Case series involving surgical options for treatment of moyamoya disease Author, Year No. of Patients Principal Procedure Mesiwala et al., 2008 39 STA-MCA bypass in 86% of ops Tripathi et al., 2007

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Jodi L. Smith

M oyamoya disease is an unremitting cerebrovascular occlusive disorder of unknown etiology that is becoming more widely recognized as a cause of stroke in pediatric and adult patients. In fact, moyamoya disease accounts for ≥ 6% of strokes in children. 73 , 96 If left untreated, it can lead to devastating, permanent neurological disability. It is characterized by progressive narrowing (and, ultimately, occlusion) of the terminal ICA and proximal middle and anterior cerebral arteries and the nearly simultaneous formation of dilated intracranial ICA

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Achal S. Achrol, Raphael Guzman, Marco Lee and Gary K. Steinberg

I n this review, we discuss recent investigations in moyamoya disease, including stem cell involvement in the pathophysiology of moyamoya disease and genetic factors underlying the etiology of moyamoya disease. We also discuss future directions, which promise not only to offer new insights into the pathogenesis of moyamoya disease but to enhance our understanding of new vessel formation in the central nervous system as it relates to stroke, vascular anomalies and tumor growth as well. Moyamoya Disease Epidemiology Originally thought to affect

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Meredith V. Olds, Robert W. Griebel, Harold J. Hoffman, Marilyn Craven, Sylvester Chuang and Hart Schutz

(STA's) to the underlying brain, which is normally supplied by the middle cerebral arteries (MCA's). Pathogenesis and Treatment of Moyamoya Disease The etiology of moyamoya disease remains unknown despite the fact that autopsies have been described for five children and 30 adults afflicted with the disorder. 3, 7, 21, 23, 35 The reported pathological findings were identical to those in two patients who underwent post-mortem examination at the Hospital for Sick Children (HSC). On gross inspection, there were infarctions of different ages, occlusions of the