Introduction: We performed 4-year follow-up neuropsychological assessment to investigate cognitive decline and the prognostic abilities from presymptomatic to symptomatic familial frontotemporal dementia (FTD). Methods: Presymptomatic MAPT (n = 15) and GRN mutation carriers (n = 31), and healthy controls (n = 39) underwent neuropsychological assessment every 2 years. Eight mutation carriers (5 MAPT, 3 GRN) became symptomatic. We investigated cognitive decline with multilevel regression modeling; the prognostic performance was assessed with ROC analyses and stepwise logistic regression. Results: MAPT converters declined on language, attention, executive function, social cognition, and memory, and GRN converters declined on attention and executive function (p < 0.05). Cognitive decline in ScreeLing phonology (p = 0.046) and letter fluency (p = 0.046) were predictive for conversion to non-fluent variant PPA, and decline on categorical fluency (p = 0.025) for an underlying MAPT mutation. Discussion: Using longitudinal neuropsychological assessment, we detected a mutation-specific pattern of cognitive decline, potentially suggesting prognostic value of neuropsychological trajectories in conversion to symptomatic FTD.

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Keywords Biomarkers, Cognition, Familial, Frontotemporal dementia, Longitudinal, Neuropsychological assessment, Presymptomatic
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Journal Journal of Neurology: official journal of the European Neurological Society
Jiskoot, L.C, Panman, J.L. (Jessica L.), van Asseldonk, L. (Lauren), Franzen, S. (Sanne), Meeter, L.H.H, Donker Kaat, L, … Papma, J.M. (2018). Longitudinal cognitive biomarkers predicting symptom onset in presymptomatic frontotemporal dementia. Journal of Neurology: official journal of the European Neurological Society, 1–12. doi:10.1007/s00415-018-8850-7