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
Frontotemporal lobar degeneration(FTLD)represents a group of clinically,neuropathologically and genetically heterogeneous disorders with plenty of overlaps between the neurodegenerative mechanism and the clinical phen...Frontotemporal lobar degeneration(FTLD)represents a group of clinically,neuropathologically and genetically heterogeneous disorders with plenty of overlaps between the neurodegenerative mechanism and the clinical phenotype.FTLD is pathologically characterized by the frontal and temporal lobar atrophy.Frontotemporal dementia(FTD)clinically presents with abnormalities of behavior and personality and language impairments variants.The clinical spectrum of FTD encompasses distinct canonical syndromes:behavioural variant of FTD(bvFTD)and primary progressive aphasia.The later includes nonfluent/agrammatic variant PPA(nfvPPA or PNFA),semantic variant PPA(svPPA or SD)and logopenic variant PPA(lvPPA).In addition,there is also overlap of FTD with motor neuron disease(FTD-MND or FTD-ALS),as well as the parkinsonian syndromes,progressive supranuclear palsy(PSP)and corticobasal syndrome(CBS).The FTLD spectrum disorders are based upon the predominant neuropathological proteins(containing inclusions of hyperphosphorylated tau or ubiquitin protein,e.g transactive response(TAR)DNA-binding protein 43 kDa(TDP-43)and fusedin-sarcoma protein in neurons and glial cells)into three main categories:(1)microtubule-associated protein tau(FTLD-Tau);(2)TAR DNA-binding protein-43(FTLD-TDP);and(3)fused in sarcoma protein(FTLD-FUS).There are five main genes mutations leading clinical and pathological variants in FTLD that identified by molecular genetic studies,which are chromosome 9 open reading frame 72(C9ORF72)gene,granulin(GRN)gene,microtubule associated protein tau gene(MAPT),the gene encoding valosin-containing protein(VCP)and the charged multivesicular body protein 2B(CHMP2B).In this review,recent advances on the different clinic variants,neuroimaging,genetics,pathological subtypes and clinicopathological associations of FTD will be discussed.展开更多
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.
基金This work was supported by the grants from the National Natural Science Foundation of China(81200991)Outstanding Young Persons’Research Program for Higher Education of Fujian Province,China(JA10123)Major Project of Fujian Science and Technology Bureau(2009D061).
文摘Frontotemporal lobar degeneration(FTLD)represents a group of clinically,neuropathologically and genetically heterogeneous disorders with plenty of overlaps between the neurodegenerative mechanism and the clinical phenotype.FTLD is pathologically characterized by the frontal and temporal lobar atrophy.Frontotemporal dementia(FTD)clinically presents with abnormalities of behavior and personality and language impairments variants.The clinical spectrum of FTD encompasses distinct canonical syndromes:behavioural variant of FTD(bvFTD)and primary progressive aphasia.The later includes nonfluent/agrammatic variant PPA(nfvPPA or PNFA),semantic variant PPA(svPPA or SD)and logopenic variant PPA(lvPPA).In addition,there is also overlap of FTD with motor neuron disease(FTD-MND or FTD-ALS),as well as the parkinsonian syndromes,progressive supranuclear palsy(PSP)and corticobasal syndrome(CBS).The FTLD spectrum disorders are based upon the predominant neuropathological proteins(containing inclusions of hyperphosphorylated tau or ubiquitin protein,e.g transactive response(TAR)DNA-binding protein 43 kDa(TDP-43)and fusedin-sarcoma protein in neurons and glial cells)into three main categories:(1)microtubule-associated protein tau(FTLD-Tau);(2)TAR DNA-binding protein-43(FTLD-TDP);and(3)fused in sarcoma protein(FTLD-FUS).There are five main genes mutations leading clinical and pathological variants in FTLD that identified by molecular genetic studies,which are chromosome 9 open reading frame 72(C9ORF72)gene,granulin(GRN)gene,microtubule associated protein tau gene(MAPT),the gene encoding valosin-containing protein(VCP)and the charged multivesicular body protein 2B(CHMP2B).In this review,recent advances on the different clinic variants,neuroimaging,genetics,pathological subtypes and clinicopathological associations of FTD will be discussed.
