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哪些特征可提高进行性核上性麻痹-帕金森综合征(PSP-P)的临床诊断准确性?

What features improve the accuracy of the clinical diagnosis of progressive supranuclear palsy-parkinsonism (PSP-P)?

机构信息

Van Cleef Roet Centre for Nervous Diseases, Monash University, Melbourne, Victoria, Australia.

出版信息

Mov Disord. 2010 Feb 15;25(3):357-62. doi: 10.1002/mds.22977.

Abstract

Progressive supranuclear palsy-parkinsonism (PSP-P) is a primary tauopathy characterised by neurofibrillary degeneration, which is frequently mistaken for Parkinson's disease (PD), multiple system atrophy (MSA), and vascular parkinsonism (VP) at presentation. The aim of this study was to identify particular clinical features (green flags) that may be helpful in differentiating PSP-P from these other disorders. We identified 37 patients with PSP-P from 726 patients archived at the Queen Square Brain Bank. Using a retrospective case notes review the clinical features were compared between the PSP-P group and Lewy body associated parkinsonism (PD, n = 444 and dementia with Lewy bodies (DLB), n = 46), MSA (n = 90), and VP (n = 19), using the chi(2)-test for proportions for a two-by-two contingency table. The sensitivity, specificity, and positive predictive values (PPV) and negative predictive values (NPV) were calculated for individual clinical features. A specificity of >0.85 or a PPV of >0.85 were considered reliable discriminators. No clinical features were predictive of PSP-P, but late drug induced dyskinesias (specificity 0.92, PPV 0.99), late autonomic dysfunction (specificity 0.94, PPV 0.99) and any visual hallucinations (specificity 0.94, PPV 0.99) were better in distinguishing PD and PSP-P than predicted using operational diagnostic criteria for PD. PSP-P shares many clinical features with PD and DLB, MSA and VP, but visual hallucinations, drug induced dyskinesias and autonomic dysfunction are very uncommon and may be helpful exclusion criteria.

摘要

进行性核上性麻痹-帕金森病(PSP-P)是一种以神经纤维变性为特征的主要 tau 病,在发病时经常被误诊为帕金森病(PD)、多系统萎缩(MSA)和血管性帕金森病(VP)。本研究的目的是确定特定的临床特征(绿色标志),这些特征可能有助于将 PSP-P 与这些其他疾病区分开来。我们从位于皇后广场脑库的 726 名患者中确定了 37 名 PSP-P 患者。使用回顾性病历回顾,使用卡方检验比较 PSP-P 组与路易体相关帕金森病(PD,n=444 例)和路易体痴呆(DLB,n=46 例)、MSA(n=90 例)和 VP(n=19 例)之间的临床特征。对于 2x2 列联表,计算了个体临床特征的敏感性、特异性和阳性预测值(PPV)和阴性预测值(NPV)。将特异性>0.85 或 PPV>0.85 视为可靠的鉴别标准。没有任何临床特征可预测 PSP-P,但迟发性药物诱导的运动障碍(特异性 0.92,PPV 0.99)、迟发性自主神经功能障碍(特异性 0.94,PPV 0.99)和任何视觉幻觉(特异性 0.94,PPV 0.99)在区分 PD 和 PSP-P 方面优于使用 PD 操作性诊断标准预测的情况。PSP-P 与 PD 和 DLB、MSA 和 VP 有许多共同的临床特征,但视觉幻觉、药物诱导的运动障碍和自主神经功能障碍非常罕见,可能是有用的排除标准。

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