After soliciting articles for this issue, we were struck by the broad range of submitted titles. Articles by J. Smith and Bendok et al. provide broad overviews of thediseaseand its manifestations. Steinberg et al. provide a thorough description of the information currently available on the origins and genetics ofmoyamoyadisease. In other articles, Derdeyn et al., Lee et al., and Bacigaluppi et al. discuss the differing imaging and cerebral blood flow techniques that are available to evaluate the patient withmoyamoyadisease, and Spetzler et al. give a
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Kiyohiro Houkin, Hiroshi Abe, Tetsuyuki Yoshimoto, and Akihiro Takahashi
T ypicalmoyamoyadiseaseis 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 ofmoyamoyadiseaserequire bilateral stenoocclusive changes verified by conventional angiography, especially in adult patients. 15 Whether “unilateral”moyamoyadisease, confirmed by typical angiographic evidence ofmoyamoyadisease单方面和正常或装备ivocal findings contralaterally, is an early form of the typical (bilateral)moyamoya
Gordon M. Burke, Allan M. Burke, Arun K. Sherma, Michael C. Hurley, H. Hunt Batjer, and Bernard R. Bendok
M oyamoyadiseaseis a chronic, occlusive cerebrovasculardiseaseinvolving bilateral stenosis or occlusion of the terminal portion of the ICAs and/or the proximal portions of the ACAs and MCAs. 60Moyamoyadiseaseis also characterized by irregular perforating vascular networks, calledmoyamoyavessels, 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 thediseaseits name, “moyamoya” in
Namio Kodama and Jiro Suzuki
T here have been few reported cases ofmoyamoyadisease, 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 inmoyamoyadisease. Case Reports Case 1 This 16-year-old girl suddenly commenced vomiting on December 24, 1968, followed by loss of consciousness. On admission, the
Moyamoya disease in monovular twins
Case report
Hiroshi Yamada, Shigetoshi Nakamura, and Naoki Kageyama
O nce considered to be a specifically Japanese entity, 6moyamoyadiseaseor syndrome has been described in patients of other races. 1, 5, 10, 12, 14, 15 Two types ofmoyamoyasyndrome 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 themoyamoyasyndrome in Japan. He found seven instances of familial occurrence among 363 cases ofmoyamoyadisease. Kitahara, et al
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 ofmoyamoyadiseaseusually includes repeated transient ischemic attacks in children and intracranial hemorrhage in adults, particularly subarachnoid hemorrhage (SAH). 8, 14 However, in the majority of cases ofmoyamoyadisease生产颅内出血,电脑mography (CT) has shown that the bleeding is intracerebral or intraventricular; therefore, whethermoyamoyadiseasedoes cause primary hemorrhage into the subarachnoid space has become doubtful. In this paper, we discuss the site of hemorrhage and the
Ali A. Baaj, Siviero Agazzi, Zafar A. Sayed, Maria Toledo, Robert F. Spetzler, and Harry van Loveren
M oyamoyadiseaseis a progressive, occlusivediseaseof the distal ICAs associated with secondary stenosis of the circle of Willis. The associated tuft of collateral vessels that forms at the base of the skull gives the angiographic appearance of a hazy “puff of smoke,” or “moyamoya” in Japanese. 39 First described in 1957 by Takeuchi and Shimizu, 42 thediseasewas initially thought to be endemic only to Japan and Asia. Thedisease, however, is also present in Europe and the Western hemisphere, although at a much lower prevalence. 48 The classic
Mamoru Tomida, Masaaki Muraki, and Kenji Yamasaki
M oyamoyadisease—spontaneous occlusion of the circle of Willis—is progressive. It is generally believed that in childrenmoyamoyadiseaseadvances in angiographically visible stages as classified by Suzuki, 12 whereas in adults thediseaserarely 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 definitemoyamoyadiseasewith drastic angiographically documented changes progressing from Suzuki
Jodi L. Smith
M oyamoyadiseaseis 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,moyamoyadiseaseaccounts 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
Achal S. Achrol, Raphael Guzman, Marco Lee, and Gary K. Steinberg
I n this review, we discuss recent investigations inmoyamoyadisease, including stem cell involvement in the pathophysiology ofmoyamoyadiseaseand genetic factors underlying the etiology ofmoyamoyadisease. We also discuss future directions, which promise not only to offer new insights into the pathogenesis ofmoyamoyadiseasebut 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.MoyamoyaDiseaseEpidemiology Originally thought to affect
