Pleasure David, Soulika Athena, Singh Sunit K, Gallo Vittorio, Bannerman Peter
Deparment of Neurology, UC Davis School of Medicine, and Shriners Hospitals for Children Northern California, Sacramento, California 95817, USA.
Ment Retard Dev Disabil Res Rev. 2006;12(2):141-6. doi: 10.1002/mrdd.20100.
While the central nervous system (CNS) is generally thought of as an immunopriviledged site, immune-mediated CNS white matter damage can occur in both the perinatal period and in adults, and can result in severe and persistent neurological deficits. Periventricular leukomalacia (PVL) is an inflammatory white matter disease of premature infants that frequently results in cerebral palsy (CP). Clinical and experimental studies show that both hypoxic/ischemic and innate immune mechanisms contribute to the destruction of immature oligodendroglia and of axons in the deep cerebral white matter in PVL. No data are yet available as to whether there is any genetic predisposition to PVL or to its neurological sequelae. Multiple sclerosis (MS) is an inflammatory white matter disease that often begins in young adulthood, causes multifocal destruction of mature oligodendroglia and of axons, and eventually leads to substantial cumulative neurological disability. Certain genetic polymorphisms contribute to susceptibility to MS, and adaptive immune responses to myelin-associated self antigens, or to exogenous antigens that mimic these self antigens, play a central role in the pathophysiology of this disease.
虽然中枢神经系统（CNS）通常被认为是一个免疫特权部位，但免疫介导的中枢神经系统白质损伤在围产期和成人期均可发生，并可导致严重且持续的神经功能缺损。脑室周围白质软化（PVL）是一种早产儿的炎症性白质疾病，常导致脑瘫（CP）。临床和实验研究表明，缺氧/缺血机制和先天免疫机制均参与了PVL中未成熟少突胶质细胞和大脑深部白质轴突的破坏。关于PVL或其神经后遗症是否存在任何遗传易感性，目前尚无数据。多发性硬化症（MS）是一种炎症性白质疾病，通常始于年轻成年期，导致成熟少突胶质细胞和轴突的多灶性破坏，并最终导致严重的累积性神经功能残疾。某些基因多态性会增加患MS的易感性，针对髓鞘相关自身抗原或模拟这些自身抗原的外源性抗原的适应性免疫反应在该疾病的病理生理学中起核心作用。
