J Korean Neurosurg Soc.  2015 Jun;57(6):408-414. 10.3340/jkns.2015.57.6.408.

Moyamoya Syndrome: A Window of Moyamoya Disease

  • 1Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. nsthomas@snu.ac.kr
  • 2Department of Anatomy, Seoul National University College of Medicine, Seoul, Korea.


Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.


Moyamoya syndrome; Neurofibromatosis; Down syndrome; Thyroid disease; Radiation

MeSH Terms

Cranial Irradiation
Down Syndrome
Moyamoya Disease*
Natural History
Neurofibromatosis 1
Thyroid Diseases
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