tau-PET 和多模态成像在以进行性核上性麻痹为表现的临床不典型多系统萎缩中的应用。

Tau-PET and multimodal imaging in clinically atypical multiple system atrophy masquerading as progressive supranuclear palsy.

机构信息

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Department of Neuroscience (Neuropathology), Mayo Clinic, Jacksonville, FL, USA.

出版信息

Parkinsonism Relat Disord. 2022 Aug;101:9-14. doi: 10.1016/j.parkreldis.2022.06.008. Epub 2022 Jun 16.

DOI:10.1016/j.parkreldis.2022.06.008

PMID:35752126

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9704374/

Abstract

INTRODUCTION

Multiple system atrophy (MSA) typically presents with parkinsonism, ataxia and/or autonomic dysfunction. Occasionally, clinically atypical (ca-MSA) cases masquerade as progressive supranuclear palsy (PSP). We aimed to investigate whether different neuroimaging modalities could facilitate differentiation and whether histopathologic characteristics could explain the atypical presentation.

METHODS

We identified 3 neuropathologically-defined ca-MSA patients with clinically diagnosed PSP who underwent various antemortem brain imaging: MRI and PET imaging using C-Pittsburgh compound B, F-flortaucipir, and F-fluorodeoxyglucose. We compared clinical features, brainstem planimetry, and radiotracer standardized uptake value ratios in ca-MSA to 10 autopsy-confirmed PSP patients and 10 healthy controls (imaging only). We also compared histologic count of neuronal loss, iron deposition and α-synuclein-immunoreactive glial cytoplasmic inclusion burden to 10 autopsy-confirmed MSA-parkinsonism (MSA-P) cases.

RESULTS

Ca-MSA had better PSP Saccadic Impairment Scale scores (p = 0.003) and more frequent good levodopa response (p = 0.061) than PSP. Ca-MSA showed higher midbrain-to-pons ratio and lower Magnetic Resonance Parkinsonism Index than PSP (each, p = 0.036) and exhibited lower glucose metabolism in the putamen and globus pallidus versus PSP (p = 0.017) and controls (p = 0.007). These same regions showed higher flortaucipir uptake in ca-MSA than PSP (p = 0.007 for putamen, p = 0.049 for pallidum) and controls (p = 0.012). Lower flortaucipir retention was observed in the subthalamic nucleus versus PSP (p = 0.007). The putamen-to-subthalamic ratio distinguished ca-MSA from PSP. No histopathological differences were observed for ca-MSA versus typical MSA-P.

CONCLUSION

Severity of saccadic impairment, levodopa responsiveness, MRI planimetric measurements, and different patterns of fluorodeoxyglucose and flortaucipir uptake can help improve antemortem differentiation of MSA masquerading as PSP from true PSP.

摘要

介绍

多系统萎缩（MSA）通常表现为帕金森病、共济失调和/或自主神经功能障碍。偶尔，临床上不典型（ca-MSA）病例会伪装为进行性核上性麻痹（PSP）。我们旨在研究不同的神经影像学模式是否有助于区分，以及组织病理学特征是否可以解释这种不典型表现。

方法

我们确定了 3 例经神经病理学定义的临床诊断为 PSP 的 ca-MSA 患者，他们接受了各种生前脑成像检查：MRI 和使用 C-Pittsburgh 化合物 B、F-氟托西匹利和 F-氟脱氧葡萄糖的 PET 成像。我们比较了 ca-MSA 与 10 例尸检证实的 PSP 患者和 10 名健康对照者（仅进行影像学检查）的临床特征、脑干平面图和放射性示踪剂标准化摄取比值。我们还比较了神经元丢失、铁沉积和α-突触核蛋白免疫反应性神经胶质细胞质包涵体负担的组织学计数与 10 例尸检证实的 MSA-帕金森病（MSA-P）病例。

结果

与 PSP 相比，ca-MSA 的 PSP 扫视障碍量表评分（p=0.003）更好，左旋多巴反应更好（p=0.061）。与 PSP 相比，ca-MSA 的中脑-桥脑比和磁共磁共振帕金森病指数（Magnetic Resonance Parkinsonism Index，MRPI）更高（均为 p=0.036），并表现出纹状体和苍白球葡萄糖代谢降低，与 PSP（p=0.017）和对照组（p=0.007）相比。在 ca-MSA 中，这些相同的区域比 PSP（纹状体 p=0.007，苍白球 p=0.049）和对照组（p=0.012）显示出更高的氟托西匹利摄取。与 PSP 相比，在 ca-MSA 中观察到Subthalamic Nucleus（黑质下核）中氟托西匹利保留率较低（p=0.007）。纹状体-黑质下核比值可将 ca-MSA 与 PSP 区分开来。与典型 MSA-P 相比，ca-MSA 无组织病理学差异。