Minimal change multiple system atrophy: An aggressive variant?
Minimal change multiple system atrophy: An aggressive variant?
复制标题
DOI：
10.1002/mds.26220
复制
发表时间：
2015-06-01
影响因子：
8.6
通讯作者：
Holton, Janice L.
中科院分区：
文献类型：
--
作者：
Ling, Helen;Asi, Yasmine T.;Holton, Janice L.
研究背景含有β-synuclein的胶质细胞包涵体是多系统萎缩（MSA）的病理标志.最小改变（MC-MSA）是一种罕见的MSA亚型，其神经元丢失主要局限于黑质和蓝斑。方法对6例MC-MSA和8例MSA对照组进行脑区免疫组化和半定量评估。结果MC-MSA组尾状核和黑质内神经元胞浆包涵体比MSA对照组多（P=0.002），在任何区域的胶质细胞胞质包涵体负载没有任何统计学差异。在两组中，在延髓腹外侧（P=1.0）和中缝隐核（P=0.4）中发现了严重的胶质细胞胞质内含物负荷。与MSA对照组相比，3例死于意外猝死的MC-MSA病例的发病年龄较早（平均年龄：38岁vs. 57.6岁，P=0.02），疾病持续时间在数值上较短（意思是：5.3 vs. 8年，P=0.2）和更快的临床进展，大多数临床里程碑在发病后3年内达到，说明是MSA的攻击性变种另外3例MC-MSA患者死于无关的并发症，发病年龄（平均：57.7岁）和时间进程与对照组相似，内侧和背外侧黑质亚区神经元丢失和胶质增生较轻（P
BackgroundGlial cytoplasmic inclusions containing -synuclein are the pathological hallmark of multiple system atrophy (MSA). Minimal change (MC-MSA) is an unusual MSA subtype with neuronal loss largely restricted to the substantia nigra and locus coeruleus.MethodsImmunohistochemistry on selected brain regions and semiquantitative assessment were performed on six MC-MSA and eight MSA control cases.ResultsMore neuronal cytoplasmic inclusions were seen in the caudate and substantia nigra in MC-MSA than in MSA controls (P=0.002), without any statistical difference in glial cytoplasmic inclusion load in any region. Severe glial cytoplasmic inclusion load was found in the ventrolateral medulla (P=1.0) and nucleus raphe obscurus (P=0.4) in both groups. When compared with MSA controls, the three MC-MSA cases who had died of sudden unexpected death had an earlier age of onset (mean: 38 vs. 57.6 y, P=0.02), a numerically shorter disease duration (mean: 5.3 vs. 8 y, P=0.2) and a more rapid clinical progression with most of the clinical milestones reached within 3 y of presentation, suggesting an aggressive variant of MSA. Another three MC-MSA cases, who had died of unrelated concurrent diseases, had an age of onset (mean: 57.7 y) and temporal course similar to controls, had less severe neuronal loss and gliosis in the medial and dorsolateral substantia nigra subregions (P