Electroencephalography (EEG) in Moyamoya Disease

  • Jong-Hee Chae
  • Ki Joong Kim


Moyamoya disease (MMD) is one of the most common causes of cerebrovascular disease in East Asian populations, especially of Japan, China and Republic of Korea. This condition is characterized by progressive stenosis of both terminal internal carotid arteries in the supracli-noid portion with the development of a network of cerebral collaterals, referred to as the moy-amoya vessels. Interestingly, clinical symptoms are different between children and adults. Children present most frequently with the symptoms of episodic cerebral ischemia like transient reversible hemiplegia, which are often precipitated by hyperventilation situation such as when crying, playing the harmonica, eating hot and spicy food, and taking a deep breath when emotionally upset, while in adults, they are intracranial hemorrhages. We can diagnose the patients by brain magnetic resonance (MR) imaging, an MR angiography, an electroencepha-lography (EEG), and a cerebral angiography without any difficulties [1–3].


Magnetic Resonance Angiography Moyamoya Disease Ischemic Hypoxia Sleep Spindle East Asian Population 
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.
    Suzuki J, Kodama N (1986) Moyamoya disease — review. Stroke 14:104–109CrossRefGoogle Scholar
  2. 2.
    Fukui M, Kono S, Sueishi K et al (2000) Moyamoya disease. Neuropathology 20(Suppl):61–64CrossRefGoogle Scholar
  3. 3.
    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
  4. 4.
    Kodama N, Aoki Y, Hiraga H et al (1979) Electroencephalographic findings in children with moyamoya disease. Arch Neurol 36:16–19PubMedCrossRefGoogle Scholar
  5. 5.
    Sunder TR, Erwin CW, Duboid PJ (1980) Hyperventilation induced abnormalities in the electroencephalogram of children with moyamoya disease. Electroencephalogr Clin Neurophysiol 49:414–420PubMedCrossRefGoogle Scholar
  6. 6.
    Konish T (1987) The standardization of hyperventilation on EEG recording in childhood II. The quantitative analysis of build-up. Brain Dev 9:21–25CrossRefGoogle Scholar
  7. 7.
    Kurlemann G, Fahrendorf G, Wolfgang K et al (1992) Characteristic EEG findings in childhood moyamoya syndrome. Neurosurg Rev 15:57–60PubMedCrossRefGoogle Scholar
  8. 8.
    Aoki Y, Hiraga H, Ichijo S (1977) EEG or the moyamoya disease. Electroencephalogr Clin Neurophysiol 43:490Google Scholar
  9. 9.
    Tagawa T, Naritomi H, Mimaki T et al (1984) Mechanism of EEG abnormality caused by hyerventilation in children with moyamoya disease-hemodynamic study be Xe 133 inhalation method. Brain Dev 6:217CrossRefGoogle Scholar
  10. 10.
    Prestwick G, Reivich M, Hill ID (1965) The EEG effects of combined hyperventilation and hypoxia in normal subjects. Electroencephalogr Clin Neurophysiol 18:56–65CrossRefGoogle Scholar
  11. 11.
    Kim DS, Ko TS, Ra YS et al (2006) Postoperative electroencephalogram for follow up of pediatric moyamoya disease. J Korean Med Sci 21:495–499PubMedCrossRefGoogle Scholar
  12. 12.
    Achenbach-NG, Mavroudakis N, Chippa KH et al (1990) Effects of routine hyperventilation on PCO2 and PO2 in normal subjects: implication form EEG interpretations. J Clin Neurophysiol 11(2):220–225Google Scholar
  13. 13.
    Kuroda S, Kamiyama H, Isobe M et al (1995) Cerebral hemodynamics and “re-build up” phenomenon on electroencephalogram in children with moyamoya disease. Childs Nerv Syst 11:214–219PubMedCrossRefGoogle Scholar
  14. 14.
    Kameyama M, Shirane R, Tsurumi Y et al (1986). Evaluation of cerebral blood flow and metabolism in childhood moyamoya disease: an investigation into “re-build up” on EEG by positron CT. Childs Nerv Syst 2:130–133PubMedCrossRefGoogle Scholar
  15. 15.
    Touho H, Karasawa J, Shishido H et al (1990) Mechanism of the re-build up phenomenon in moyamoya disease. Neurol Med Chir (Tokyo) 30:721–726CrossRefGoogle Scholar
  16. 16.
    Lin Y, Yoshiko K, Negoro T et al (2000) Cerebral oxygenation state in childhood moyamoya disease: a near-infrared spectroscopy study. Pediatr Neurol 22:365–369PubMedCrossRefGoogle Scholar
  17. 17.
    Kuroda S, Houkin K, Hoshi Y et al (1996) Cerebral hypoxia after hyperventilation causes “re-build up” phenomenon and TIA in childhood moyamoya disease: a near-infrared spectroscopy study. Childs Nerv Syst 12:448–453PubMedCrossRefGoogle Scholar
  18. 18.
    Fernandez-Fernandez S, Vazquez-Lopez M, Carrasco-Marina LL et al (2003) An association between moyamoya disease and morning glory anomaly. Rev Neurol 37:541–544PubMedGoogle Scholar
  19. 19.
    Kacinski M, Kubik A, Kroczka S et al (2007) Clinical and video-EEG findings in a girl with juvenile moyamoya disease. Brain Dev 29:603–606PubMedCrossRefGoogle Scholar
  20. 20.
    Kim HY, Chung CS, Lee J et al (2003) Hyperventilation-induced limb shaking TIA in moyamoya disease. Neurology 60:137–139PubMedCrossRefGoogle Scholar
  21. 21.
    Karasawa J, Touho H, Ohnishi H et al (1992) Long-term follow up study after extracranial intracranial bypass surgery for anterior circulation ischemia in childhood moyamoya disease. J Neurosurg 77:84–89PubMedCrossRefGoogle Scholar

Copyright information

© Springer 2010

Authors and Affiliations

  1. 1.Department of Pediatrics, Pediatric Clinical Neuroscience CenterSeoul National University Children's Hospital, Seoul National University College of MedicineJongno-guRepublic of Korea
  2. 2.Research Center for Rare DiseaseRepublic of Korea

Personalised recommendations