Takahiro Ota1, Masaki Komiyama2. 1. Department of Neurosurgery, Tokyo Metropolitan Tama Medical Center, Japan. 2. Department of Neurointervention, Osaka City General Hospital, Japan.
Abstract
BACKGROUND: The neural crest is a transient structure present in early embryogenesis. Cephalic neural crest cells migrate into the pharyngeal arches and the frontonasal process that becomes the forehead and midfacial structures. They also contribute to forming the media of the arteries of the circle of Willis and their branches. The cardiac neural crest produces vascular smooth muscle cells in the ascending aorta, cardiac septum and coronary arteries. METHODS: In this review, we evaluate the role of the neural crest in moyamoya disease and the pathological implications from the concurrence of moyamoya disease and cardiovascular diseases from the point of view of neural crest cell distributions. RESULTS: Midline craniofacial and central nervous system anomalies with eye anomalies, morning glory disc anomaly in patients with moyamoya disease can both be explained as a subtype of cephalic neurocristopathy. Further, the association between moyamoya disease and cardiac manifestations (congenital cardiac defects and coronary artery disease) have also been reported. Both the cephalic neural crest and cardiac neural crest contribute to these concurrent arterial diseases, as cardio-cephalic neurocristopathy. CONCLUSION: The concept of cephalic/cardio-cephalic neurocristopathy provides a new perspective to understanding the underlying aetiological associations and to developing future therapeutic approaches for concomitant moyamoya disease and cardiovascular diseases.
BACKGROUND: The neural crest is a transient structure present in early embryogenesis. Cephalic neural crest cells migrate into the pharyngeal arches and the frontonasal process that becomes the forehead and midfacial structures. They also contribute to forming the media of the arteries of the circle of Willis and their branches. The cardiac neural crest produces vascular smooth muscle cells in the ascending aorta, cardiac septum and coronary arteries. METHODS: In this review, we evaluate the role of the neural crest in moyamoya disease and the pathological implications from the concurrence of moyamoya disease and cardiovascular diseases from the point of view of neural crest cell distributions. RESULTS: Midline craniofacial and central nervous system anomalies with eye anomalies, morning glory disc anomaly in patients with moyamoya disease can both be explained as a subtype of cephalic neurocristopathy. Further, the association between moyamoya disease and cardiac manifestations (congenital cardiac defects and coronary artery disease) have also been reported. Both the cephalic neural crest and cardiac neural crest contribute to these concurrent arterial diseases, as cardio-cephalic neurocristopathy. CONCLUSION: The concept of cephalic/cardio-cephalic neurocristopathy provides a new perspective to understanding the underlying aetiological associations and to developing future therapeutic approaches for concomitant moyamoya disease and cardiovascular diseases.
Authors: Edward Teng; Justin Heller; Jorge Lazareff; Henry Kawamoto; Kristy Wasson; Jose I Garri; James P Bradley Journal: J Craniofac Surg Date: 2006-09 Impact factor: 1.046
Authors: Dianna M Milewicz; John R Østergaard; Leena M Ala-Kokko; Nadia Khan; Dorothy K Grange; Roberto Mendoza-Londono; Timothy J Bradley; Ann Haskins Olney; Lesley Adès; Joseph F Maher; Dongchuan Guo; L Maximilian Buja; Dong Kim; James C Hyland; Ellen S Regalado Journal: Am J Med Genet A Date: 2010-10 Impact factor: 2.802