Primary progressive aphasia presents with aphasia, with or without other minor cognitive dysfunction. It refers to core linguistic disorders caused by neurodegenerative disease. Three main PPA variants are recognized:...Primary progressive aphasia presents with aphasia, with or without other minor cognitive dysfunction. It refers to core linguistic disorders caused by neurodegenerative disease. Three main PPA variants are recognized: nonfluent/agrammatic, semantic and logopenic. Correctly classifying patients during life according to the underlying histopathology will become increasingly important as cause-specific treatments become available. This article reviews clinical and histopathological studies of PPA, with particular reference to updated PPA classifications. Currently, one-to-one relationships do not exist within PPA subtypes. The semantic variant has the best correspondence between the clinical syndrome and the underlying pathological cause and the logopenic variant the worst correspondence. The use of future biomarkers should facilitate accurate clinicopathological correlation of patients during life.展开更多
Background Primary progressive aphasia(PPA)is a neurodegenerative disorder characterized by a gradual,insidious and progressive loss of language abilities,with naming difficulties being an early and persistent impairm...Background Primary progressive aphasia(PPA)is a neurodegenerative disorder characterized by a gradual,insidious and progressive loss of language abilities,with naming difficulties being an early and persistent impairment common to all three variants.In the absence of effective pharmacological treatments and given the progressive nature of the disorder,in the past few decades,many studies have investigated the effectiveness of language training to minimize the functional impact of word-finding difficulties in daily life.Main body We review language treatments most commonly used in clinical practice among patients with different variants of PPA,with a focus on the enhancement of spoken and written naming abilities.Generalization of gains to the ability to name untrained stimuli or to other language abilities and the maintenance of these results over time are also discussed.Forty-eight studies were included in this literature review,identifying four main types of language treatment:a)lexical retrieval treatment,b)phonological and/or orthographic treatment,c)semantic treatment,and d)a multimodality approach treatment.Overall,language training is able to induce immediate improvements of naming abilities in all variants of PPA.Moreover,despite the large variability among results,generalization and long-term effects can be recorded after the training.The reviewed studies also suggest that one factor that determines the choice of a particular approach is the compromised components of the lexical/semantic processing system.Conclusion The majority of studies have demonstrated improvements of naming abilities following language treatments.Given the progressive nature of PPA,it is essential to apply language treatment in the early stages of the disease.展开更多
目的探讨核苷酸结合寡聚化结构域样受体3(NLRP3)、半胱氨酸蛋白酶-1(Caspase-1)与白细胞介素-1β(Interleukin-1β,IL-1β)水平变化在原发性膝关节骨性关节炎(Osteoarthritis of the knee,KOA)病情进展中的临床意义。方法选取武威市凉...目的探讨核苷酸结合寡聚化结构域样受体3(NLRP3)、半胱氨酸蛋白酶-1(Caspase-1)与白细胞介素-1β(Interleukin-1β,IL-1β)水平变化在原发性膝关节骨性关节炎(Osteoarthritis of the knee,KOA)病情进展中的临床意义。方法选取武威市凉州医院2021年12月至2023年7月收治的122例KOA患者为观察组,另选取同期本院收治的单纯半月板损伤且无软骨损伤的115例者作为对照组。比较两组NLRP3、Caspase-1与IL-1β水平;比较轻中度KOA患者和重度KOA患者一般资料(年龄、性别、体质量指数)、NLRP3、Caspase-1、IL-1β水平和其他炎性指标(IL-1、IL-17)的差异;采用多因素Logistic回归分析影响KOA病情进展的危险因素;采用ROC曲线分析NLRP3、Caspase-1与IL-1β水平及三者联合检测预测重度KOA的效能。结果观察组NLRP3、Caspase-1与IL-1β水平均高于对照组,差异具有统计学意义(P<0.05);经Lequesne评分评估,轻中度KOA患者90例、重度KOA患者32例。重度KOA组NLRP3、Caspase-1、IL-1βIL-1、IL-17水平高于轻中度KOA组,差异具有统计学意义(P<0.05);两组性别、年龄、体质量指数比较差异无统计学意义(P>0.05);多因素Logistic回归分析结果显示,NLRP3、Caspase-1与IL-1β水平高表达均是影响KOA病情进展的危险因素(P<0.05);ROC曲线显示,NLRP3、Caspase-1与IL-1β单独预测重度KOA的AUC为0.654、0.709、0.703,三者联合检测的AUC为0.878,明显高于各指标单独检测AUC值(P<0.05)。结论NLRP3、Caspase-1与IL-1β水平随着KOA病情进展而上升,三种指标水平变化对重度KOA具有预测作用,在KOA疾病的进展中具有潜在的评估价值。展开更多
文摘Primary progressive aphasia presents with aphasia, with or without other minor cognitive dysfunction. It refers to core linguistic disorders caused by neurodegenerative disease. Three main PPA variants are recognized: nonfluent/agrammatic, semantic and logopenic. Correctly classifying patients during life according to the underlying histopathology will become increasingly important as cause-specific treatments become available. This article reviews clinical and histopathological studies of PPA, with particular reference to updated PPA classifications. Currently, one-to-one relationships do not exist within PPA subtypes. The semantic variant has the best correspondence between the clinical syndrome and the underlying pathological cause and the logopenic variant the worst correspondence. The use of future biomarkers should facilitate accurate clinicopathological correlation of patients during life.
基金This work was supported by the Italian Ministry of Health(Ricerca Corrente and Giovani Ricercatori grant GR-2018-12365105).
文摘Background Primary progressive aphasia(PPA)is a neurodegenerative disorder characterized by a gradual,insidious and progressive loss of language abilities,with naming difficulties being an early and persistent impairment common to all three variants.In the absence of effective pharmacological treatments and given the progressive nature of the disorder,in the past few decades,many studies have investigated the effectiveness of language training to minimize the functional impact of word-finding difficulties in daily life.Main body We review language treatments most commonly used in clinical practice among patients with different variants of PPA,with a focus on the enhancement of spoken and written naming abilities.Generalization of gains to the ability to name untrained stimuli or to other language abilities and the maintenance of these results over time are also discussed.Forty-eight studies were included in this literature review,identifying four main types of language treatment:a)lexical retrieval treatment,b)phonological and/or orthographic treatment,c)semantic treatment,and d)a multimodality approach treatment.Overall,language training is able to induce immediate improvements of naming abilities in all variants of PPA.Moreover,despite the large variability among results,generalization and long-term effects can be recorded after the training.The reviewed studies also suggest that one factor that determines the choice of a particular approach is the compromised components of the lexical/semantic processing system.Conclusion The majority of studies have demonstrated improvements of naming abilities following language treatments.Given the progressive nature of PPA,it is essential to apply language treatment in the early stages of the disease.
文摘目的探讨核苷酸结合寡聚化结构域样受体3(NLRP3)、半胱氨酸蛋白酶-1(Caspase-1)与白细胞介素-1β(Interleukin-1β,IL-1β)水平变化在原发性膝关节骨性关节炎(Osteoarthritis of the knee,KOA)病情进展中的临床意义。方法选取武威市凉州医院2021年12月至2023年7月收治的122例KOA患者为观察组,另选取同期本院收治的单纯半月板损伤且无软骨损伤的115例者作为对照组。比较两组NLRP3、Caspase-1与IL-1β水平;比较轻中度KOA患者和重度KOA患者一般资料(年龄、性别、体质量指数)、NLRP3、Caspase-1、IL-1β水平和其他炎性指标(IL-1、IL-17)的差异;采用多因素Logistic回归分析影响KOA病情进展的危险因素;采用ROC曲线分析NLRP3、Caspase-1与IL-1β水平及三者联合检测预测重度KOA的效能。结果观察组NLRP3、Caspase-1与IL-1β水平均高于对照组,差异具有统计学意义(P<0.05);经Lequesne评分评估,轻中度KOA患者90例、重度KOA患者32例。重度KOA组NLRP3、Caspase-1、IL-1βIL-1、IL-17水平高于轻中度KOA组,差异具有统计学意义(P<0.05);两组性别、年龄、体质量指数比较差异无统计学意义(P>0.05);多因素Logistic回归分析结果显示,NLRP3、Caspase-1与IL-1β水平高表达均是影响KOA病情进展的危险因素(P<0.05);ROC曲线显示,NLRP3、Caspase-1与IL-1β单独预测重度KOA的AUC为0.654、0.709、0.703,三者联合检测的AUC为0.878,明显高于各指标单独检测AUC值(P<0.05)。结论NLRP3、Caspase-1与IL-1β水平随着KOA病情进展而上升,三种指标水平变化对重度KOA具有预测作用,在KOA疾病的进展中具有潜在的评估价值。
