Department of Neurosurgery, Harvard Medical School, Massachusetts General Hospital , Boston, MA , USA ; School of Clinical Medicine, Addenbrooke's Hospital, University of Cambridge , Cambridge , UK.
Front Neurol. 2013 Aug 14;4:105. doi: 10.3389/fneur.2013.00105. eCollection 2013.
Moyamoya disease is a slowly progressing steno-occlusive condition affecting the cerebrovasculature. Affecting the terminal internal carotid arteries (ICA) and there branches, bilaterally, a resulting in a fine vascular network in the base of the brain to allow for compensation of the stenosed vessels. While there is obvious evidence of the involvement of inflammatory proteins in the condition, this has historically not been acknowledged as a causal factor. Here we describe the fundamental histopathology, genetics, and signaling cascades involved in moyamoya and debate whether these factors can be linked as causal factor for the condition or whether they are simply a secondary result of the ischemia described in the condition. A particular focus has been placed on the multitude of signaling cascades linked to the condition as these are viewed as having the greatest therapeutic potential. As such we hope to draw some novel insight into potential diagnostic and therapeutic inflammatory targets in the condition.
烟雾病是一种缓慢进展的狭窄性闭塞性脑血管疾病。影响颈内动脉末端（ICA）及其分支，双侧受累，导致脑底形成精细的血管网，以代偿狭窄的血管。虽然有明显的证据表明炎症蛋白参与了这种疾病，但这在历史上并未被认为是一个因果因素。在这里，我们描述了烟雾病的基本组织病理学、遗传学和信号级联反应，并探讨了这些因素是否可以作为该疾病的因果因素，或者它们是否仅仅是该疾病中描述的缺血的一个次要结果。特别关注与该疾病相关的多种信号级联反应，因为这些反应被认为具有最大的治疗潜力。因此，我们希望对该疾病的潜在诊断和治疗炎症靶点有一些新的认识。
