Moyamoya Syndrome: A Window of Moyamoya Disease.
10.3340/jkns.2015.57.6.408
- Author:
Ji Hoon PHI
1
;
Kyu Chang WANG
;
Ji Yeoun LEE
;
Seung Ki KIM
Author Information
1. Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. nsthomas@snu.ac.kr
- Publication Type:Review
- Keywords:
Moyamoya syndrome;
Neurofibromatosis;
Down syndrome;
Thyroid disease;
Radiation
- MeSH:
Cranial Irradiation;
Down Syndrome;
Humans;
Moyamoya Disease*;
Natural History;
Neurofibromatoses;
Neurofibromatosis 1;
Thyroid Diseases
- From:Journal of Korean Neurosurgical Society
2015;57(6):408-414
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
