Dr. Hiren Pandya, Dr. Prasanna Patel, Dr. Kanaiya Patel, Dr. Harsh Pathak


Moyamoya disease is a chronic, progressive occlusion of the circle of Willis arteries that leads to the development of characteristic collateral vessels seen on imaging, particularly cerebral angiography. The disease may develop in children and adults, but the clinical features differ. Moyamoya disease occurs predominantly in Japanese individuals but has been found in all races with varying age distributions and clinical manifestations. As a result, moyamoya disease has been underrecognized as a cause of ischemic and hemorrhagic strokes.

Moyamoya disease was first described in Japan by Takeuchi and Shimizu in 1957.[1] Although the disease is most common in Japan, many subsequent cases have been reported elsewhere, including North America and Europe.[2-3] 

Moyamoya disease is deemed a progressive steno-occlusive disease at terminal portions of the bilateral internal carotid arteries with the development of “moyamoya vessels” as collateral channels of circulation.[4] The appearance of these small, multiple vessels at the base of the brain on catheter angiography was originally described by the Japanese term moyamoya, which translates to “puff of smoke.”[5]

Full Text:



Takeuchi K, Shimizu K. Hypogenesis of bilateral internal carotid arteries [in Japanese]. No To Shinkei. 1957;9:37-43.

Chiu D, Shedden P, Bratina P, Grotta JC. Clinical features of moyamoya disease in the United States. Stroke. 1998;29:1347-1351. http://stroke.ahajournals.org/cgi/content/full/29/7/1347. Accessed August 27, 2009.

Bertora P, Lovati C, Gambaro P, Vicenzi A, Rosa S, Osio M, et al. Moyamoya disease in a member of the Roma gypsy community [letter] [published online ahead of print February 8, 2008]. Eur Neurol. 2008:59:274-275. http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=000115643. Accessed August 27, 2009.

Kitamura K, Fukui M, Oka K. Moyamoya disease. In: Handbook of Clinical Neurology. Vol2 . Amsterdam, The Netherlands: Elsevier;1989 : 293-306.

Suzuki J, Takaku A. Cerebrovascular `moyamoya' disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969;20:288-299.

Miyamoto S, Yoshimoto T, Hashimoto N, et al.: Effects of extracranial-intracranial bypass for patients with hemorrhagic moyamoya disease: results of the Japan Adult Moyamoya Trial. Stroke. 2014, 45:1415-1421. 10.1161/STROKEAHA.113.004386

Huang S, Guo ZN, Shi M, Yang Y, Rao M: Etiology and pathogenesis of Moyamoya disease: an update on disease prevalence. Int J Stroke. 2017, 12:246-253. 10.1177/1747493017694393

Scott RM, Smith ER: Moyamoya disease and Moyamoya syndrome. N Engl J Med. 2009, 360:1226-1237. 10.1056/nejmra0804622

Janda PH, Bellew JG, Veerappan V: Moyamoya disease: case report and literature review. J Am Osteopath Assoc. 2009; 109(10): 547–53. PubMed Abstract

Han DH, Kwon OK, Byun BJ, Choi BY, Choi CW, Choi JU, et al. A co-operative study: Clinical characteristics of 334 Korean patients with moyamoya disease treated at neurosurgical institutes (1976-1994). The Korean Society for Cerebrovascular Disease. Acta Neurochir (Wien) 2000;142:1263-73.

Smith ER, Scott RM. Surgical management of moyamoya syndrome. Skull Base 2005;15:15-26.


  • There are currently no refbacks.