Jiskoot LC, et al. - Experts examined the cognitive decline and the prognostic abilities from presymptomatic to symptomatic familial frontotemporal dementia (FTD) in a 4-year follow-up neuropsychological analysis. A mutation-specific pattern of cognitive decline was identified via longitudinal neuropsychological assessment. This indicated a potential prognostic value of neuropsychological trajectories in conversion to symptomatic FTD.

Methods

• Neuropsychological assessment was carried out in presymptomatic MAPT (n = 15) and GRN mutation carriers (n = 31), and healthy controls (n = 39) every 2 years.
• A total of 8 mutation carriers (5 MAPT, 3 GRN) became symptomatic.
• Cognitive decline via multilevel regression modeling was evaluated, along with the prognostic performance via ROC analyses and stepwise logistic regression.

Results

• A decline was reported in the MAPT converters on language, attention, executive function, social cognition, and memory, while GRN converters declined on attention and executive function (p <0.05).
• As per the outcomes, cognitive decline in ScreeLing phonology (p=0.046) and letter fluency (p=0.046) predicted conversion to non-fluent variant PPA and decline on categorical fluency (p=0.025) for an underlying MAPT mutation.