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Prevalence of cerebrospinal fluid Alzheimer disease-like pattern in atypical dementias
Article Number: SIS807143AI201222
20th December, 2022
Author(s):
A. Padovani1 , A. Benussi1 , F. Ferrari1 , S. Archetti1,2, B. Borroni1*
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 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.