Shi Jiong, Baxter Leslie C, Kuniyoshi Sandra M
Departments of *Neurology †Neuropsychology and Neuroradiology, Barrow Neurological Institute, Phoenix, AZ ‡Department of Neuroscience, Arizona State University, Phoenix, AZ.
Cogn Behav Neurol. 2014 Mar;27(1):1-7. doi: 10.1097/WNN.0000000000000023.
From 1868, when Charcot first described the clinical features and the pathologic correlates, up till the present day, multiple sclerosis (MS) has commonly been characterized by the symptoms caused by inflammatory plaques in the white matter of the brain and spinal cord. Early use of magnetic resonance imaging (MRI) to diagnose MS focused on detecting these white matter lesions. By the 1990s, researchers recognized that many patients with MS have cognitive deficits that can cause severe disability, and also determined the associated pathology; these findings shed more light on both the pathogenesis and progression. Since 2004, several lines of evidence have shown that the extent of white matter plaques identified on MRI does not correlate well with cognitive deficits. High-resolution MRI and advances in immunohistochemical techniques have enabled detection of cortical demyelination early in the course, correlating with cognitive deficits. Late in the course, pathologic changes in normal-looking white and gray matter correlate more closely with progressive cognitive deficits than with visual, sensory, and motor symptoms. This finding implies the need to redefine the disease and its progression. In this review, we discuss the histopathologic studies of cortical plaques in MS and early indications about their role in disease definition and progression, describe the role of high-resolution MRI in staging and determining progression of cognitive symptoms, and discuss how advances in these areas are forcing us to rethink diagnosis and determination of progression.
从1868年夏科首次描述多发性硬化症（MS）的临床特征及病理关联至今，MS通常表现为脑和脊髓白质炎性斑块所引发的症状。早期利用磁共振成像（MRI）诊断MS主要聚焦于检测这些白质病变。到了20世纪90年代，研究人员认识到许多MS患者存在可导致严重残疾的认知缺陷，并确定了相关病理；这些发现为发病机制和疾病进展提供了更多线索。自2004年以来，多项证据表明，MRI上识别出的白质斑块范围与认知缺陷的相关性不佳。高分辨率MRI和免疫组织化学技术的进步使得在病程早期就能检测到皮质脱髓鞘，这与认知缺陷相关。在病程后期，外观正常的白质和灰质中的病理变化与进行性认知缺陷的相关性比与视觉、感觉和运动症状的相关性更为密切。这一发现意味着需要重新定义该疾病及其进展。在本综述中，我们讨论了MS中皮质斑块的组织病理学研究及其在疾病定义和进展中的作用的早期迹象，描述了高分辨率MRI在认知症状分期和确定进展方面的作用，并讨论了这些领域的进展如何迫使我们重新思考诊断和进展的判定。
