Long-standing multiple system atrophy-Parkinsonism with limbic and FTLD-type α-synuclein pathology.
Long-standing multiple system atrophy-Parkinsonism with limbic and FTLD-type α-synuclein pathology.
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DOI：
10.1111/nan.12783
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发表时间：
2022-04
影响因子：
5
通讯作者：
Hiniker, Annie
中科院分区：
文献类型：
--
作者：
Coughlin, David G.;Dryden, Ian;Goodwill, Vanessa S.;Pizzo, Donald P.;Wright, Brenton;Lessig, Stephanie;Galasko, Douglas;MacKenzie, Ian R.;Hiniker, Annie
多系统萎缩(MSA)是一种散发性神经退行性疾病，临床表现为自主神经功能衰竭、不同程度的帕金森病和小脑性共济失调。平均发病年龄为60岁，存活5年，尽管已描述了暴发性和更良性的病程(1)。MSA的病理特征是在少突胶质细胞(胶质胞质包涵体)和神经元(神经元胞质包涵体和神经元核包涵体)中存在丰富的α-Synuclein阳性蛋白聚集体。GCIS密度与病程和神经元丢失的严重程度相关(2)。在这里，我们提出了一个罕见的MSA病例，其临床特征是迅速下降，然后在严重感染状态下持续20年的病程。在尸检中，大脑显示出严重的胶质细胞增生和神经元丢失，这是MSA的特征。此外，还可见不典型的Pick体样和环状α突触核蛋白包涵体，多见于边缘区域，与最近描述的MSA亚型“非典型MSA”一致(3)。四种不同的α-突触核蛋白抗体仅突出了少量α-突触核蛋白阳性的病理；然而，
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease clinically marked by autonomic failure and variable degrees of parkinsonism and cerebellar ataxias. The average age of onset is in the sixth decade of life with a five-year survival, although fulminant and more benign courses have been described (1). The pathologic hallmark of MSA is abundant α-synuclein-positive protein aggregates within oligodendrocytes (glial cytoplasmic inclusions, GCI) and neurons (neuronal cytoplasmic inclusions, NCI, and neuronal nuclear inclusions, NNI). The density of GCIs has been correlated with disease duration and severity of neuronal loss (2). Here, we present an unusual case of MSA clinically characterized by rapid decline followed by a 20-year disease duration in a severely affected state. At autopsy, the brain showed severe gliosis and neuronal loss in a pattern characteristic of MSA. Additionally, there were atypical Pick body-like and ring-shaped α-synuclein inclusions, most prominent in limbic regions, consistent with a recently described subtype of MSA designated “atypical MSA”(3). Four different α-synuclein antibodies highlighted only scant α-synuclein-positive pathology; however,
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影响因子：
16.6
作者：
Choi, Insup;Zhang, Yuanxi;Yue, Zhenyu
通讯作者：
Yue, Zhenyu
影响因子：
6.1
作者：
Chu, Yaping;Dodiya, Hemraj;Kordower, Jeffrey H.
通讯作者：
Kordower, Jeffrey H.
影响因子：
14.5
作者：
Ozawa, T;Paviour, D;Revesz, T
通讯作者：
Revesz, T
影响因子：
9.9
作者：
Gilman, S.;Wenning, G. K.;Vidailhet, M.
通讯作者：
Vidailhet, M.
神经病理学中的银诊断：原理，实践和修订的解释。
影响因子：
12.7
作者：
Uchihara, Toshiki
通讯作者：
Uchihara, Toshiki