Clinical Features of Moyamoya Disease: An Overview

  • Yong-Seung Hwang


The two cardinal clinical features of moyamoya disease are ischemic attacks or intracranial hemorrhages. However, the main clinical presentations of moyamoya disease differ substantially between children and adults. Most children with moyamoya disease develop transient ischemic attacks (TIA) or cerebral infarction, whereas about half of the adult patients develop intracranial hemorrhage, and half develop TIA or cerebral infarction, or both [1]. Regarding the ethnic difference, clinical features and the course of moyamoya disease in whites clearly differ from moyamoya disease in Asians in the timing of the onset of vasculopathy and lower rate of hemorrhage [2].


Cerebral Infarction Transient Ischemic Attack Basilar Artery Intracranial Aneurysm Moyamoya Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Kuroda S, Houkin K (2008) Moyamoya disease: current concepts and future perspectives. Lancet Neurol 7:1056–1066PubMedCrossRefGoogle Scholar
  2. 2.
    Kraemer M, Heienbrok W, Berlit P (2008) Moyamoya disease in Europeans. Stroke 39:3193–3200PubMedCrossRefGoogle Scholar
  3. 3.
    Fukui M, Kono S, Sueishi K et al (2000) Moyamoya disease. Neuropathology 20:S61–S64PubMedCrossRefGoogle Scholar
  4. 4.
    Mikulis DJ, Krolczyk G, Desal H et al (2005) Preoperative and postoperative mapping of cerebrovascular reactivity in moyamoya disease by using blood oxygen level-dependent magnetic resonance imaging. J Neurosurg 103:347–355PubMedCrossRefGoogle Scholar
  5. 5.
    Kim S, Seol HJ, Cho B et al (2004) Moyamoya disease among young patients: its aggressive clinical course and the role of active surgical treatment. Neurosurgery 54:840–846PubMedCrossRefGoogle Scholar
  6. 6.
    Suzuki J, Kodama N (1983) Moyamoya disease — a review. Stroke 14:104–109PubMedCrossRefGoogle Scholar
  7. 7.
    Irikura K, Miyasaka Y, Kurata I et al (1996) A source of haemorrhage in adult patients with moyamoya disease: the significance of tributaries from the choroidal artery. Acta Neurochir (Wien) 138:1282–1286CrossRefGoogle Scholar
  8. 8.
    Kawaguchi S, Sakaki T, Morimoto T et al (1996) Characteristics of intracranial aneurysms associated with moyamoya disease. A review of 111 cases. Acta Neurochir (Wien) 138:1287–1294CrossRefGoogle Scholar
  9. 9.
    Marushima A, Yanaka K, Matsuki T et al (2006) Subarachnoid hemorrhage not due to ruptured aneurysm in moyamoya disease. J Clin Neurosci 13:146–149PubMedCrossRefGoogle Scholar
  10. 10.
    Osanai T, Kuroda S, Nakayama N et al (2008) Moyamoya disease presenting with subarachnoid hemorrhage localized over the frontal cortex: case report. Surg Neurol 69:197–200PubMedCrossRefGoogle Scholar
  11. 11.
    Yonekawa Y, Kahn N (2003) Moyamoya disease. Adv Neurol 92:113–118PubMedGoogle Scholar
  12. 12.
    Manceau E, Giroud M, Dumas R (1997) Moyamoya disease in children. A review of the clinical and radiological features and current treatment. Childs Nerv Syst 13:595–600PubMedCrossRefGoogle Scholar
  13. 13.
    Nakase H, Ohnishi H, Touho H et al (1993) Long-term follow-up study of “epileptic type” moyamoya disease in children. Neurol Med Chir (Tokyo) 33:621–624CrossRefGoogle Scholar
  14. 14.
    Kikuta K, Takagi Y, Arakawa Y et al (2006) Absence epilepsy associated with moyamoya disease. Case report. J Neurosurg (4 Suppl Pediatrics) 104:265–268PubMedGoogle Scholar
  15. 15.
    Moritake K, Handa H, Yonekawa Y et al (1986) Follow-up study on the relationship between age at onset of illness and outcome in patients with moyamoya disease. No Shinkei Geka 14:957–963PubMedGoogle Scholar
  16. 16.
    Hirano T, Uyama E, Tashima K et al (1998) An atypical case of adult moyamoya disease with initial onset of brain stem ischemia. J Neurol Sci 157:100–104PubMedCrossRefGoogle Scholar

Copyright information

© Springer 2010

Authors and Affiliations

  1. 1.Department of PediatricsPediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul National University College of MedicineJongno-guRepublic of Korea

Personalised recommendations