Moyamoya disease is a rare vascular disorder in which the terminal branches of the internal carotid artery progressively narrow1.
Epidemiology
In Japan, the annual incidence is 0.35 to 0.94 per 100,000, with a prevalence of 3.16 to 10.5 per 100,0002 3. In the United States, the incidence is lower at 0.086 per 100,000 inhabitants, but higher in the Asian American population at 0.28 per 100,0004.
Imaging
An ivy sign may be found on MRI in some cases.
Classification
The disease stage can be classified using the Suzuki classification.
Treatment
Depending on the disease stage, anatomy, and clinical symptoms, bypass surgery may be considered in patients with moyamoya disease.
Histopathology
In moyamoya disease, the vascular stenosis is caused by hyperplasia of the smooth muscle. The intima is typically eccentrically thickened, and the internal elastic lamina is tortuous or duplicated5.
References
Kuroda S, Houkin K. Moyamoya disease: current concepts and future perspectives. The Lancet Neurology. 2008;7(11):1056-1066. ↩ ↩
Wakai, Kenji, et al. "Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey." Clinical neurology and neurosurgery 99 (1997): S1-S5. ↩ ↩
Achrol, Achal S., et al. "Pathophysiology and genetic factors in moyamoya disease." Neurosurgical focus 26.4 (2009): E4. ↩ ↩
Kuroda, Satoshi, and Yoshinobu Iwasaki. "Current review of familial moyamoya disease." Nihon rinsho. Japanese journal of clinical medicine 64 (2006): 750-754. ↩ ↩
Neurosurgery Case Review: Questions and Answers. Remi Nader, Abdulrahman J Sabbagh. 2020 Thieme Verlage. ISBN 9781626231986 ↩ ↩