BACKGROUND: Differential diagnosis between Frontotemporal Dementia (FTD), Corticobasal Syndrome (CBS), Progressive Supranuclear Palsy Syndrome (PSP), FTD with motor neuron disease (FTD-MND) is often challenging, becau...BACKGROUND: Differential diagnosis between Frontotemporal Dementia (FTD), Corticobasal Syndrome (CBS), Progressive Supranuclear Palsy Syndrome (PSP), FTD with motor neuron disease (FTD-MND) is often challenging, because of the occurrence of atypical cases. Autopsy series have identified Alzheimer Disease (AD) pathology in a consistent percentage of patients with atypical dementias. It has been demonstrated that Cerebrospinal Fluid (CSF) Tau/Aβ42 dosage is a reliable marker for AD. OBJECTIVE: To evaluate the presence and percentage of CSF AD-like patterns (high CSF tau/Aβ42 ratio) in patients with atypical dementias in order to identify an ongoing AD neurodegenerative process. METHODS: One hundred seventy two consecutive patients fulfilling current clinical criteria for behavioural variant FTD (bvFTD, n = 73), agrammatic variant of Primary Progressive Aphasia (avPPA, n = 19), semantic variant of PPA (svPPA, n = 12), FTD-MND (n = 5), CBS (n = 42), PSP (n = 21) were recruited and underwent CSF analysis. CSF AD-like and non AD (nAD-like) patterns were identified. RESULTS: CSF AD-like pattern was reported in 6 out of 73 cases (8.2%) in the bvFTD group, in 3 out of 19 (15.8%) in the avPPA group, and in 7 out of 42 (16.7%) in the CBS group. One out of 12 (8.3%) of svPPA had CSF AD-like pattern. None of patients FTD-MND and PSP had CSF AD-like pattern. No differences in demographic characteristics were detected between subgroups in each phenotype. CONCLUSIONS: Our findings convey that the CSF tau/ Aβ42 ratio could be found in a proportion of cases with clinical bvFTD, avPPA and CBD. Detecting anon-going AD pathological process in atypical dementias has several implications for defining distinctive therapeutic approaches, guiding genetic screening and helping in patients’ selection in future clinical trials.展开更多
Transient Global Amnesia (TGA) is a common condition of unknown aetiology char acterised by the abrupt onset of severe anterograde amnesia, which lasts less th an 24 hours. Some authors have suggested that subclinical...Transient Global Amnesia (TGA) is a common condition of unknown aetiology char acterised by the abrupt onset of severe anterograde amnesia, which lasts less th an 24 hours. Some authors have suggested that subclinical impairment of memory f unctions may persist for much longer, but neuropsychological assessment lasting years after TGA attack has not been performed so far. The aim of this study was to evaluate long term cognitive functions in patients with a previous TGA episo de. Fifty five patients underwent a standardised neuropsychological assessment after at least one year from the TGA attack, and were compared with 80 age mat ched controls. TGA patients showed worse performances on tests evaluating verbal and nonverbal long term memory and attention, with comparable global cognitive functions. By applying current criteria for amnestic Mild Cognitive Impairment (MCI a) on TGA subjects, a group consisting of 18/55 (32.7%) MCI a subjects w as identified. There was no association between the presence of MCI a and demog raphic variables, vascular risk factors, years since the TGA episode, or ApoE ge notype. This study demonstrates that TGA appears to be a relatively benign syndr ome although objective memory deficits ffulilling MCI a criteria persist over t ime, as detected by multidimensional neuropsychological tasks performed at long term follow up.展开更多
文摘BACKGROUND: Differential diagnosis between Frontotemporal Dementia (FTD), Corticobasal Syndrome (CBS), Progressive Supranuclear Palsy Syndrome (PSP), FTD with motor neuron disease (FTD-MND) is often challenging, because of the occurrence of atypical cases. Autopsy series have identified Alzheimer Disease (AD) pathology in a consistent percentage of patients with atypical dementias. It has been demonstrated that Cerebrospinal Fluid (CSF) Tau/Aβ42 dosage is a reliable marker for AD. OBJECTIVE: To evaluate the presence and percentage of CSF AD-like patterns (high CSF tau/Aβ42 ratio) in patients with atypical dementias in order to identify an ongoing AD neurodegenerative process. METHODS: One hundred seventy two consecutive patients fulfilling current clinical criteria for behavioural variant FTD (bvFTD, n = 73), agrammatic variant of Primary Progressive Aphasia (avPPA, n = 19), semantic variant of PPA (svPPA, n = 12), FTD-MND (n = 5), CBS (n = 42), PSP (n = 21) were recruited and underwent CSF analysis. CSF AD-like and non AD (nAD-like) patterns were identified. RESULTS: CSF AD-like pattern was reported in 6 out of 73 cases (8.2%) in the bvFTD group, in 3 out of 19 (15.8%) in the avPPA group, and in 7 out of 42 (16.7%) in the CBS group. One out of 12 (8.3%) of svPPA had CSF AD-like pattern. None of patients FTD-MND and PSP had CSF AD-like pattern. No differences in demographic characteristics were detected between subgroups in each phenotype. CONCLUSIONS: Our findings convey that the CSF tau/ Aβ42 ratio could be found in a proportion of cases with clinical bvFTD, avPPA and CBD. Detecting anon-going AD pathological process in atypical dementias has several implications for defining distinctive therapeutic approaches, guiding genetic screening and helping in patients’ selection in future clinical trials.
文摘Transient Global Amnesia (TGA) is a common condition of unknown aetiology char acterised by the abrupt onset of severe anterograde amnesia, which lasts less th an 24 hours. Some authors have suggested that subclinical impairment of memory f unctions may persist for much longer, but neuropsychological assessment lasting years after TGA attack has not been performed so far. The aim of this study was to evaluate long term cognitive functions in patients with a previous TGA episo de. Fifty five patients underwent a standardised neuropsychological assessment after at least one year from the TGA attack, and were compared with 80 age mat ched controls. TGA patients showed worse performances on tests evaluating verbal and nonverbal long term memory and attention, with comparable global cognitive functions. By applying current criteria for amnestic Mild Cognitive Impairment (MCI a) on TGA subjects, a group consisting of 18/55 (32.7%) MCI a subjects w as identified. There was no association between the presence of MCI a and demog raphic variables, vascular risk factors, years since the TGA episode, or ApoE ge notype. This study demonstrates that TGA appears to be a relatively benign syndr ome although objective memory deficits ffulilling MCI a criteria persist over t ime, as detected by multidimensional neuropsychological tasks performed at long term follow up.
