Widespread demyelination in the cerebellar cortex in multiple sclerosis
Widespread demyelination in the cerebellar cortex in multiple sclerosis
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DOI：
10.1111/j.1750-3639.2006.00041.x
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发表时间：
2007-01-01
期刊：
影响因子：
6.4
通讯作者：
Lassmann, Hans
中科院分区：
文献类型：
--
作者：
Kutzelnigg, Alexandra;Faber-Rod, Jens C.;Lassmann, Hans
前脑新皮质脱髓鞘最近被确定为多发性硬化症（MS）的重要病理特征。在这里，我们描述小脑皮质是脱髓鞘的主要好发部位，特别是对于原发性和继发性进行性多发性硬化症患者。在这些患者中，平均 38.7% 的小脑皮质区域受到影响，极端情况下可达 92%。小脑皮质脱髓鞘主要以带状方式发生，影响多个叶。病变的特点是原发性脱髓鞘，轴突和神经元相对保留，尽管存在一些轴突球体和浦肯野细胞中度减少。虽然皮质脱髓鞘有时与邻近白质脱髓鞘（白质病变）同时发生，但在大多数情况下，皮质受到的影响独立于白质病变。我们发现皮质和白质脱髓鞘之间没有相关性，并且在某些情况下，在几乎没有白质病变的情况下存在广泛的皮质脱髓鞘。我们的数据表明皮质脱髓鞘是多发性硬化症小脑功能障碍的潜在基础。
Neocortical demyelination in the forebrain has recently been identified as an important pathological feature of multiple sclerosis (MS). Here we describe that the cerebellar cortex is a major predilection site for demyelination, in particular in patients with primary and secondary progressive MS. In these patients, on average, 38.7% of cerebellar cortical area is affected, reaching in extreme examples up to 92%. Cerebellar cortical demyelination occurs mainly in a band-like manner, affecting multiple folia. The lesions are characterized by primary demyelination with relative axonal and neuronal preservation, although some axonal spheroids and a moderate reduction of Purkinje cells are present. Although cortical demyelination sometimes occurs together with demyelination in the adjacent white matter (leukocortical lesions), in most instances, the cortex was affected independently from white matter lesions. We found no correlation between demyelination in the cortex and the white matter, and in some cases, extensive cortical demyelination was present in the near absence of white matter lesions. Our data identify cortical demyelination as a potential substrate of cerebellar dysfunction in MS.