Rapid Cognitive Deterioration in Progressive Supranuclear Palsy: A 1-Year Follow-Up Study.

IF 2.6 4区 医学 Q2 CLINICAL NEUROLOGY
Xin-Yi Li, Yu-Jie Yang, Fang-Yang Jiao, Gan Tang, Ming-Jia Chen, Rui-Xin Yao, Yi-Xin Zhao, Xiao-Niu Liang, Bo Shen, Yi-Min Sun, Jian-Jun Wu, Jian Wang, Feng-Tao Liu
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引用次数: 0

Abstract

Background: Nowadays, cognitive impairment has been characterized as one of the most vital clinical symptoms in progressive supranuclear palsy (PSP).

Objectives: Based on a relatively large cohort, we aimed to show the cognitive deterioration in different PSP subtypes during 1-year follow-up and investigate potential contributors for disease prognosis.

Methods: One hundred seventeen patients from Progressive Supranuclear Palsy Neuroimage Initiative (PSPNI) cohort underwent neuropsychological tests and 1-year follow-up, with 73 diagnosed as PSP-Richardson syndrome (PSP-RS) and 44 as PSP-non-RS. Patients were divided into normal cognition (PSP-NC), mild cognitive impairment (PSP-MCI), and PSP-dementia. Cognitive impairment and progression rates were compared between PSP-RS and PSP-non-RS, and determinants for MCI conversion to dementia were calculated by multiple cox regression.

Results: At baseline, 30.8% of PSP patients were diagnosed as dementia, 53.0% as MCI, and only 16.2% as NC. Compared to PSP-non-RS, PSP-RS suffered more from motor symptoms and cognitive impairment. During follow-up, PSP-RS also exhibited faster disease progression in Mini-Mental State Examination and visuospatial function, with cognitive deterioration in attention and executive function, but retained in language and memory subdomains. Twenty-seven of 62 PSP-MCI patients converted to dementia during follow-up, with the diagnosis of RS subtype as the most significant contributor to conversion (hazard ration = 2.993, 95% confidence interval = 1.451, 5.232, P = 0.009).

Conclusions: Patients with PSP-RS showed more severe cognitive impairment and faster decline longitudinally than patients with PSP-non-RS. Additionally, the diagnosis of RS subtype appears to be the most contributed factor for MCI conversion to dementia within just 1-year follow-up period.

进行性核上性麻痹患者认知能力快速下降:1年随访研究。
背景:目前,认知障碍已被认为是进行性核上性麻痹(PSP)最重要的临床症状之一。目的:基于一个相对较大的队列,我们的目的是在1年的随访中显示不同PSP亚型的认知退化,并研究疾病预后的潜在影响因素。方法:进行性核上性麻痹神经影像倡议(PSPNI)队列117例患者进行神经心理测试和1年随访,其中73例诊断为psp -理查德森综合征(PSP-RS), 44例诊断为psp -非rs。患者分为正常认知(PSP-NC)、轻度认知障碍(PSP-MCI)和psp -痴呆。比较PSP-RS和psp -非rs之间的认知障碍和进展率,并通过多重cox回归计算MCI转化为痴呆的决定因素。结果:在基线时,30.8%的PSP患者被诊断为痴呆,53.0%被诊断为MCI,只有16.2%被诊断为NC。与PSP-non-RS相比,PSP-RS有更多的运动症状和认知障碍。在随访期间,PSP-RS在迷你精神状态检查和视觉空间功能方面也表现出更快的疾病进展,在注意和执行功能方面表现出认知衰退,但在语言和记忆子领域中保持不变。62例PSP-MCI患者中有27例在随访期间转化为痴呆,其中RS亚型的诊断是最显著的转化因素(风险比= 2.993,95%可信区间= 1.451,5.232,P = 0.009)。结论:PSP-RS患者认知功能障碍较psp -非rs患者更为严重,纵向衰退速度更快。此外,RS亚型的诊断似乎是MCI在短短1年随访期内转化为痴呆的最重要因素。
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来源期刊
CiteScore
4.00
自引率
7.50%
发文量
218
期刊介绍: Movement Disorders Clinical Practice- is an online-only journal committed to publishing high quality peer reviewed articles related to clinical aspects of movement disorders which broadly include phenomenology (interesting case/case series/rarities), investigative (for e.g- genetics, imaging), translational (phenotype-genotype or other) and treatment aspects (clinical guidelines, diagnostic and treatment algorithms)
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