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A decreased cerebrospinal fluid (CSF) p‐Tau 181  to total tau ratio (p/t‐tau) is a biomarker for frontotemporal lobar degeneration with TDP43 inclusions (FTLD‐TDP) and for amyotrophic lateral sclerosis (ALS). CSF light chain neurofilaments (NfL) are increased in ALS. We examined whether CSF p/t‐tau and NfL are related to ALS status in FTLD‐TDP.    Methods  We compared CSF p/t‐tau and NfL levels between patients with FTLD‐TDP with ALS (n = 15), FTLD‐TDP without ALS (n = 17), FTLD‐Tau (n = 6), Alzheimer's disease (AD; n = 25), and subjective memory complaints (SMC, n = 24).    Results  Apart from FTLD‐Tau, all groups differed significantly with increasing p/t‐tau ratios from FTLD‐TDP with ALS to FTLD‐TDP without ALS to AD and SMC. CSF NfL was very high in FTLD‐TDP with ALS followed by FTLD‐TDP without ALS, AD, and SMC. Both biomarkers correlated with survival.    Discussion  CSF p/t‐tau ratio and NfL levels are strongly driven by ALS status. These markers, therefore, appear to be more of prognostic than diagnostic significance.

Discriminative and prognostic potential of cerebrospinal fluid phosphoTau/tau ratio and neurofilaments for frontotemporal dementia subtypes