Atypical multiple system atrophy is a new subtype of frontotemporal lobar degeneration: frontotemporal lobar degeneration associated with α-synuclein

被引:0
作者
Naoya Aoki
Philip J. Boyer
Cheryl Lund
Wen-Lang Lin
Shunsuke Koga
Owen A. Ross
Myron Weiner
Anne Lipton
James M. Powers
Charles L. White
Dennis W. Dickson
机构
[1] Mayo Clinic,Department of Neuroscience
[2] University of Colorado,Department of Pathology
[3] Venice Regional Hospital,Department of Anesthesiology
[4] University of Texas Southwestern Medical Center,Department of Psychiatry and Neurology
[5] University of Rochester Medical Center,Department of Pathology
[6] University of Texas Southwestern Medical Center,Department of Pathology
来源
Acta Neuropathologica | 2015年 / 130卷
关键词
Multiple system atrophy; Frontotemporal lobar degeneration; Neuropathology; Pick body-like inclusions; α-Synuclein;
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学科分类号
摘要
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease clinically characterized by cerebellar signs, parkinsonism, and autonomic dysfunction. Pathologically, MSA is an α-synucleinopathy affecting striatonigral and olivopontocerebellar systems, while neocortical and limbic involvement is usually minimal. In this study, we describe four patients with atypical MSA with clinical features consistent with frontotemporal dementia (FTD), including two with corticobasal syndrome, one with progressive non-fluent aphasia, and one with behavioral variant FTD. None had autonomic dysfunction. All had frontotemporal atrophy and severe limbic α-synuclein neuronal pathology. The neuronal inclusions were heterogeneous, but included Pick body-like inclusions. The latter were strongly associated with neuronal loss in the hippocampus and amygdala. Unlike typical Pick bodies, the neuronal inclusions were positive on Gallyas silver stain and negative on tau immunohistochemistry. In comparison to 34 typical MSA cases, atypical MSA had significantly more neuronal inclusions in anteromedial temporal lobe and limbic structures. While uncommon, our findings suggest that MSA may present clinically and pathologically as a frontotemporal lobar degeneration (FTLD). We suggest that this may represent a novel subtype of FTLD associated with α-synuclein (FTLD-synuclein).
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页码:93 / 105
页数:12
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