• Saad Ullah Temple Univerity/Conemaugh Valley Memorial Hospital, Johnstown, PA
  • Anas Ahmed Siddiqui Dow Medical College, Karachi
  • Waseem Zaid Alkilani Temple Univerity/Conemaugh Valley Memorial Hospital, Johnstown, PA
  • Noman Ahmed Jang Khan Temple Univerity/Conemaugh Valley Memorial Hospital, Johnstown, PA
  • Hafiz Umair Siddiqui Dow Medical College, Karachi


Moyamoya disease is an idiopathic progressive vasculopathy of distal internal carotid artery and circle of Willis which leads to the development of characteristic smoky appearance of the vascular collateral network on angiography. With the highest reported incidence among Japanese population, it has been under recognized as a cause of cerebrovascular accidents in Western countries. Here we report a case of a young 20-year-old Caucasian woman who presented to the emergency department with expressive aphasia, right arm weakness and numbness for three days. Imaging modalities confirmed Moyamoya disease.Keywords:  internal carotid artery; circle of Willis; cerebral angiography


Takeuchi K, Shimizu K. Hypogenesis of bilateral internal carotid arteries. No To Shinkei 1957;9:37–43.

Suzuki J, Takaku A. Cerebrovascular `moyamoya' disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol 1969;20:288–99.

Kitamura K, Fukui M, Oka K. Moyamoya disease. In: Handbook of Clinical Neurology. Vol 2. Amsterdam, The Netherlands: Elsevier; 1989. p.293–306.

Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, et al. Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg 1997;99(Suppl 2):S1–5.

Uchino K, Johnston SC, Becker KJ, Tirschwell DL. Moyamoya disease in Washington State and California. Neurology 2005;65(6):956–8.

Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 2008;79(8):900–4.

Yamashita M, Oka K, Tanaka K. Histopathology of the brain vascular network in moyamoya disease. Stroke 1983;14(1):50–8.

Ikeda E. Systemic vascular changes in spontaneous occlusion of the circle of Willis. Stroke 1991;22(11):1358–62.

Hoare AM, Keogh AJ. Cerebrovascular moyamoya disease. Br Med J 1974;1(5905):430–2.

Fukui M. Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (“moyamoya” disease). Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya disease) of the Ministry of Health and Welfare, Japan. Clin Neurol Neurosurg 1997;99(Suppl 2):S238–40.

Spittler JF, Smektala K. Pharmacotherapy in moyamoya disease. Hokkaido Igaku Zasshi 1990;65(2):235–40.

Fung LW, Thompson D, Ganesan V. Revascularization surgery for pediatric moyamoya: a review of the literature. Childs Nerv Syst 2005;21(5):358–64.

Fukui M. Current state of study on moyamoya disease in Japan. Surg Neurol 1997;47(2):138–43.

Imaizumi T, Hayashi K, Saito K, Osawa M, Fukuyama Y. Long-term outcomes of pediatric moyamoya disease monitored to adulthood. Pediatr Neurol 1998;18(4):321–5.