Language training for nonfluent aphasia （NFA） patients rmay increase their verbal expression of unfamiliarwords. Some therapies aimed at the improvement of cognitive functions can facilitate the recovery of NFA pati...Language training for nonfluent aphasia （NFA） patients rmay increase their verbal expression of unfamiliarwords. Some therapies aimed at the improvement of cognitive functions can facilitate the recovery of NFA patients＇ damaged linguistic functions. Some studies have shown that with music cues NFA patients could fluently sing their familiar songs but could not read the lyrics, consistent with studies of proverbs and prayer） Our previous research has shown that highly related voice cues can improve NFA patients＇ verbal expression.2 These results indicate that the improvement of NFA patients＇ speech production may benefit from regaining the extraction of phonological encoding that has already been preserved in memory, rather than re-study of the language.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
文摘Language training for nonfluent aphasia （NFA） patients rmay increase their verbal expression of unfamiliarwords. Some therapies aimed at the improvement of cognitive functions can facilitate the recovery of NFA patients＇ damaged linguistic functions. Some studies have shown that with music cues NFA patients could fluently sing their familiar songs but could not read the lyrics, consistent with studies of proverbs and prayer） Our previous research has shown that highly related voice cues can improve NFA patients＇ verbal expression.2 These results indicate that the improvement of NFA patients＇ speech production may benefit from regaining the extraction of phonological encoding that has already been preserved in memory, rather than re-study of the language.
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.
