{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T19:17:42Z","timestamp":1790709462599,"version":"4.1.0"},"reference-count":156,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FOC"],"published-print":{"date-parts":[[2009,4]]},"abstract":"<jats:p>Moyamoya, meaning a \u201chazy puff of smoke\u201d in Japanese, is a chronic, occlusive cerebrovascular disease involving bilateral stenosis or occlusion of the terminal portion of the internal carotid arteries (ICAs) and\/or the proximal portions of the anterior cerebral arteries and middle cerebral arteries (MCAs). The Ministry of Health and Welfare of Japan has defined 4 types of moyamoya disease (MMD): ischemic, hemorrhagic, epileptic, and \u201cother.\u201d The ischemic type has been shown to predominate in childhood, while the hemorrhagic type is more often observed in the adult population. The highest prevalence of MMD is found in Japan, with a higher female to male ratio. Studies have shown a possible genetic association of MMD linked to chromosome 17 in Japanese cases as well as in cases found in other demographics. During autopsy, intracerebral hematoma is found and most commonly serves as the major cause of death in patients with MMD. Moyamoya vessels at the base of the brain are composed of medium-sized or small muscular arteries emanating from the circle of Willis, mainly the intracranial portions of ICAs, anterior choroidal arteries, and posterior cerebral arteries, forming complex channels that connect with distal positions of the MCAs. Off of these channels are small tortuous and dilated vessels that penetrate into the base of the brain at the site of the thalamoperforate and lenticulostriate arteries. On angiography, there is the characteristic stenosis or occlusion bilaterally at the terminal portion of the ICAs as well as the moyamoya vessels at the base of the brain. Six angiographic stages have been described, from Stage 1, which reveals a narrowing of the carotid forks, to Stage 6, in which the moyamoya vessels disappear and collateral circulation is produced solely from the external carotid arteries. Cases with milder symptoms are usually treated conservatively; however, more severe symptomatic cases are treated using revascularization procedures. Surgical treatments are divided into 3 types: direct, indirect, and combined\/other methods. Direct bypass includes superficial temporal artery-MCA bypass or use of other graft types. Indirect procedures bring in circulation to the intracranial regions by introducing newly developed vasculature from newly approximated tissues. These procedures may not be enough to prevent further ischemia; therefore, a combination of direct and indirect procedures is more suitable. This article will give a review of the epidemiology, natural history, pathology, pathophysiology, and diagnostic criteria, including imaging, and briefly describe the surgical treatment of MMD.<\/jats:p>","DOI":"10.3171\/2009.1.focus08310","type":"journal-article","created":{"date-parts":[[2009,3,31]],"date-time":"2009-03-31T12:12:42Z","timestamp":1238501562000},"page":"E11","source":"Crossref","is-referenced-by-count":171,"title":["Moyamoya disease: a summary"],"prefix":"10.3171","volume":"26","author":[{"given":"Gordon M.","family":"Burke","sequence":"first","affiliation":[{"name":"New York Medical College, Valhalla, New York; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allan M.","family":"Burke","sequence":"additional","affiliation":[{"name":"Departments of Neurology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arun K.","family":"Sherma","sequence":"additional","affiliation":[{"name":"Neurological Surgery, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael C.","family":"Hurley","sequence":"additional","affiliation":[{"name":"Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. Hunt","family":"Batjer","sequence":"additional","affiliation":[{"name":"Neurological Surgery, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernard R.","family":"Bendok","sequence":"additional","affiliation":[{"name":"Neurological Surgery, and"},{"name":"Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-0260011","series-title":"Neurosurg Clin N Am","first-page":"613","article-title":"Preview of a new trial of extracranial-to-intracranial arterial anastomosis: the carotid occlusion surgery study","volume":"12","author":"Adams","year":"2001"},{"key":"b2-0260011","series-title":"No Shinkei Geka","first-page":"305","article-title":"[Usefulness and limitation of MR imaging and MR angiography in diagnosis of juvenile moyamoya disease.]","volume":"21","author":"Aoki","year":"1993"},{"issue":"2 Suppl","key":"b3-0260011","series-title":"Clin Neurol Neurosurg","first-page":"S213","article-title":"Development of intimal thickening in superficial temporal arteries in patients with moyamoya disease","volume":"99","author":"Aoyagi","year":"1997"},{"key":"b4-0260011","series-title":"J Neurol Neurosurg Psychiatry","first-page":"900","article-title":"Novel epidemiological features of moyamoya disease","volume":"79","author":"Baba","year":"2008"},{"key":"b5-0260011","series-title":"Stroke","first-page":"1786","article-title":"Prothrombotic disorders in children with moyamoya syndrome","volume":"32","author":"Bonduel","year":"2001"},{"key":"b6-0260011","series-title":"Zh Vopr Neirokhir Im N N Burdenko","first-page":"11","article-title":"[Combined treatment for Moya-Moya disease, by using direct anastomosis and revascularization: experience of 225 operations.]","volume":"3","author":"Byval'tsev","year":"2007"},{"key":"b7-0260011","series-title":"Stroke","first-page":"2810","article-title":"MR perfusion imaging in Moyamoya Syndrome: potential implications for clinical evaluation of occlusive cerebrovascular disease","volume":"32","author":"Calamante","year":"2001"},{"key":"b8-0260011","series-title":"AJNR Am J Neuroradiol","first-page":"822","article-title":"Intracranial time-resolved contrast-enhanced MR angiography at 3T","volume":"27","author":"Cashen","year":"2006"},{"key":"b9-0260011","series-title":"J Neurol Neurosurg Psychiatry","first-page":"289","article-title":"Moya-moya disease: clinical and pathological report of a case with associated myopathy","volume":"42","author":"Coakham","year":"1979"},{"key":"b10-0260011","series-title":"AJNR Am J Neuroradiol","first-page":"1228","article-title":"Arterial spin-labeling in routine clinical practice, part 1: technique and artifacts","volume":"29","author":"Deibler","year":"2008"},{"key":"b11-0260011","series-title":"J Nucl Med","first-page":"2030","article-title":"Dual-isotope brain SPECT imaging with technetium-99m and iodine-123: validation by phantom studies","volume":"33","author":"Devous","year":"1992"},{"key":"b12-0260011","series-title":"N Engl J Med","first-page":"1191","article-title":"Failure of extracranial-intracranial arterial bypass to reduce the risk of ischemic stroke. 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Suppl","key":"b3-0260011","series-title":"Clin Neurol Neurosurg","first-page":"S213","article-title":"Development of intimal thickening in superficial temporal arteries in patients with moyamoya disease","volume":"99","author":"Aoyagi","year":"1997"},{"key":"b4-0260011","series-title":"J Neurol Neurosurg Psychiatry","first-page":"900","article-title":"Novel epidemiological features of moyamoya disease","volume":"79","author":"Baba","year":"2008"},{"key":"b5-0260011","series-title":"Stroke","first-page":"1786","article-title":"Prothrombotic disorders in children with moyamoya syndrome","volume":"32","author":"Bonduel","year":"2001"},{"key":"b6-0260011","series-title":"Zh Vopr Neirokhir Im N N Burdenko","first-page":"11","article-title":"[Combined treatment for Moya-Moya disease, by using direct anastomosis and revascularization: experience of 225 operations.]","volume":"3","author":"Byval'tsev","year":"2007"},{"key":"b7-0260011","series-title":"Stroke","first-page":"2810","article-title":"MR perfusion imaging in Moyamoya Syndrome: potential implications for clinical evaluation of occlusive cerebrovascular disease","volume":"32","author":"Calamante","year":"2001"},{"key":"b8-0260011","series-title":"AJNR Am J Neuroradiol","first-page":"822","article-title":"Intracranial time-resolved contrast-enhanced MR angiography at 3T","volume":"27","author":"Cashen","year":"2006"},{"key":"b9-0260011","series-title":"J Neurol Neurosurg Psychiatry","first-page":"289","article-title":"Moya-moya disease: clinical and pathological report of a case with associated myopathy","volume":"42","author":"Coakham","year":"1979"},{"key":"b10-0260011","series-title":"AJNR Am J Neuroradiol","first-page":"1228","article-title":"Arterial spin-labeling in routine clinical practice, part 1: technique and 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