Abstract
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.
Highlights
One of the current issues in the spectrum of atypical dementias is the correspondence between phenotypic features and molecular pathology
Cerebrospinal Fluid (CSF) Alzheimer Disease (AD)-like pattern was observed in 15.8% (3/19) of agrammatic variant of PPA (avPPA) and in 16.7% (7/42) of Corticobasal Syndrome (CBS) patients
We investigated the presence and the percentage of CSF AD-like pattern in patients with atypical dementias
Summary
One of the current issues in the spectrum of atypical dementias is the correspondence between phenotypic features and molecular pathology. As behavioural variant Frontotemporal Dementia (bvFTD), semantic variant of primary Progressive Aphasia (svPPA) and agrammatic variant of PPA (avPPA), FTD with Motor Neuron Disease (FTDMND), Corticobasal Syndrome (CBS), and Progressive Supranuclear Palsy (PSP), are clinically and neuropathologically heterogeneous disorders [1,2]. The phenotypic overlap between neurodegenerative disorders has been established by neuropathological studies, demonstrating that AD pathology can be found in a significant percentage of patients with alternative clinical diagnosis, as in atypical dementias [5,6]. 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. Detecting an on-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
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