Table 2

Demographic characteristics, including age, education, sex, site of onset, time from onset to baseline, familial/sporadic family history and C9orf72 status at each time point

Participant characteristicsTime 1 (n=189)Time 2 (n=117)Time 3 (n=80)Time 4 (n=49)
Age, mean years (SD)63.59 (11.42)61.64 (12.09)60.52 (12.64)58.94 (12.57)
Education, mean years (SD)13.8 (3.82)13.97 (3.41)14.28 (3.36)13.94 (2.93)
Sex, n



 Male119775434
 Female70402715
Site of onset, n


 Bulbar4424124
 Spinal145936945
Time from onset to assessment, mean months (SD)19.48 (13.29)25.4 (15.1)30.11 (14.74)34.53 (15.52)
ALSFRS-R, mean (SD)36.5 (7.6)33.43 (7.84)32.34 (8.29)31.4 (8.63)
Delta ALSFRS-R, change in ALSFRS-R per month0.520.230.21
Familial/sporadic, n


 Familial31231713
 Sporadic158946336
C9orf72 status, n


 Positive151164
 Negative1741067545
El escorial diagnostic status, n


 Definite92503323
 Probable59442914
 Possible38231812
Strong criteria status, n


 ALSn140896743
 ALSci331995
 ALSbi6210
 ALScbi10731
  • ALS, amyotrophic lateral sclerosis; ALSbi, ALS with behavioural impairment; ALScbi, ALS with cognitive and behavioural impairment; ALSci, ALS with cognitive impairment; ALSFRS-R, ALS Functional Rating Scale -Revised; ALSn, ALS with normal cognition and behaviour.