Table 2

Baseline FRS scores according to CDR+NACC FTLD severity level, by genetic group

CDR+NACC FTLD Global Group GRN mutation carriers MAPT mutation carriers C9orf72 mutation carriersControls
00.5≥100.5≥100.5≥1
N1142548421322923269268
FRS % Score97.4 (5.7)90.9† (14.2)46.3*, †, ‡ (28.1)93.0 (11.2)89.7 (18.9)43.0*, †, ‡ (27.1)94.9 (8.2)85.0*, † (20.2)32.7*, †, ‡ (25.0)96.2 (7.7)
CDR+NACC FTLD Sum of Boxes0.0 (0.0)1.0*, † (0.8)9.7*, †, ‡ (6.2)0.0 (0.0)1.1 (0.8)*, †10.5*, †, ‡ (5.9)0.0 (0.0)1.1*, † (0.7)11.1*, †, ‡ (5.5)0.2 (0.6)
  • Values are mean (SD) unless stated.

  • CDR+NACC FTLD=CDR Dementia Staging Instrument plus Behaviour and Language domains from the NACC FTLD module.

  • CDR+NACC FTLD sum of boxes scores are also shown in the same groups for comparison.

  • *P<0.05 versus controls.

  • †P<0.05 versus CDR+NACC-FTLD Global score=0 group (within the same genetic group).

  • ‡P<0.05 versus CDR+NACC-FTLD Global score=0.5 group (within the same genetic group).

  • CDR, Clinical Dementia Rating; FRS, Frontotemporal dementia Rating Scale; FTLD, Frontotemporal Lobar Degeneration; NACC, National Alzheimer’s Disease Coordinating Center.