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弥漫性特发性桥脑神经胶质瘤病(diffuse idiopathic brainstem gliosis,DIPG)合并 PSP-FTD 复合体:一种可能的 PSP 变异型。

PSP-FTD Complex: A Possible Variant of PSP.

机构信息

Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

出版信息

Am J Alzheimers Dis Other Demen. 2020 Jan-Dec;35:1533317520922383. doi: 10.1177/1533317520922383.

Abstract

INTRODUCTION

This study tried to find out type of lobar features found in patients with progressive supranuclear palsy (PSP) and whether they differ from those of frontotemporal dementia (FTD) as both of these are tauopathies.

METHODS

We studied lobar functions of 45 patients with PSP.

RESULTS

Five (11.1%) patients had no lobar feature; 11 (24.4%) had PSP-like features like apathy, frontal release signs, impaired motor Luria written sequences, and fist-edge-palm test; and 29 (64.4%) patients had FTD-like lobar features like disinhibition, poor naming, and word finding difficulty. Among features resembling FTD, behavioural variant type occurred in 31.1%, primary progressive aphasia type occurred in 58.6%, 3.4% patients had semantic dementia type features, and 6.9% were unclassified.

CONCLUSIONS

Hence, patients with PSP with lobar features may fall in the middle of PSP-FTD spectrum with frontal lobe features typical of PSP (PSP-frontal like) and those with frontal lobe features resembling FTD (PSP-FTD complex) in between.

摘要

简介

本研究试图找出进行性核上性麻痹(PSP)患者的脑叶特征类型,以及这些特征是否与同为tau 蛋白病的额颞叶痴呆(FTD)不同。

方法

我们研究了 45 例 PSP 患者的脑叶功能。

结果

5 例(11.1%)患者无脑叶特征;11 例(24.4%)具有 PSP 样特征,如冷漠、额叶释放征、运动性 Luria 书写序列受损和握拳-对掌试验;29 例(64.4%)患者具有 FTD 样脑叶特征,如行为障碍、命名障碍和找词困难。在类似于 FTD 的特征中,行为变异型占 31.1%,原发性进行性失语型占 58.6%,3.4%患者具有语义性痴呆型特征,6.9%患者无法分类。

结论

因此,具有脑叶特征的 PSP 患者可能位于 PSP-FTD 谱系的中间,具有典型 PSP 额叶特征(PSP-额叶样)和具有类似于 FTD 的额叶特征(PSP-FTD 复合)。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0fe5/10623927/0d94fb2800e9/10.1177_1533317520922383-fig1.jpg

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