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. Author manuscript; available in PMC: 2024 Jul 1.
Published in final edited form as: Alzheimers Dement. 2023 Jan 2;19(7):2842–2852. doi: 10.1002/alz.12898

Fig. 1.

Fig. 1.

EC and PT scores reflect disease severity measured by the global CDR® plus NACC FTLD score regardless of pathogenic variant status, and worsen at a similar rate over time in pathogenic variant carriers and non-carriers.

(A) LME model analysis in the full sample (n=594) revealed a significant main effect of CDR® plus NACC FTLD (p<0.001) with regard to the EC score, showing that the score changes as a function of disease stage from asymptomatic to very mild (estimate=4.38, 95%CI=[2.79, 5.97], p<0.001), very mild to mild (estimate=−3.52, 95%CI=[−5.19, −1.84], p<0.001), and mild to moderate/severe (estimate=2.93, 95%CI=[1.71, 4.14], p<0.001) disease. The interaction between CDR® plus NACC FTLD and pathogenic variant status did not reach statistical significance. (B) Similar to the EC subscale, CDR® plus NACC FTLD significantly predicted the PT score (p<0.001), showing that the score significantly worsened between asymptomatic and very mild (estimate=5.64, 95%CI=[3.81, 7.48], p<0.001), very mild and mild (estimate=−3.24, 95%CI=[−4.82, −1.13], p=0.002), as well as between mild and moderate/severe (estimate=1.49, 95%CI=[0.18, 2.79], p=0.023) disease stage. The interaction CDR® plus NACC FTLD by disease stage at baseline did not reach statistical significance for predicting the PT score. (C) In the fully longitudinal sample (n=91), disease duration significantly predicted (estimate=−0.29, 95%CI=[−0.59, −0.00], p=0.049) the EC score, demonstrating that patients with longer disease duration had lower EC score compared to patients with shorter disease duration. However, the interaction disease duration by pathogenic variant status was not statistically significant. (D) The PT score was significantly predicted by disease duration (estimate=−0.49, 95%CI=[−0.90, −0.09], p=0.019), showing that the score significantly decreased over time with longer disease duration. The interaction between disease duration and pathogenic variant status did not reach statistical significance for predicting the PT score. Age at symptom onset and sex were included as covariates of no interest in each analysis. CDR® plus NACC FTLD=CDR® Dementia Staging Instrument plus Behavior and Language domains from the NACC FTLD Module.