TABLE 2

Baseline Penn cohort characteristics by composite outcome (including death)

CharacteristicComposite outcomep-value
No outcome (n=465)Outcome achieved (n=300)
Age at diagnosis years61±1265±11<0.001
Male sex272 (59)168 (56)0.50
Ethnicity0.25
 Caucasian410 (88)252 (84)
 African-american28 (6)25 (8)
 Other27 (6)23 (8)
BMI class kg·m−2<0.001
 <18.59 (2)21 (7)
 18.5–24.9186 (40)132 (44)
 25–29.9177 (38)93 (31)
 ≥3093 (20)54 (18)
Diagnosis delay years1.0 (0.6–2.0)0.8 (0.5–1.3)<0.001
El Escorial criteria<0.001
 Definite ALS62 (13)90 (30)
 Possible ALS125 (27)72 (24)
 Probable ALS154 (33)85 (28)
 Suspected ALS124 (27)53 (18)
Symptom onset site0.002
 Limb380 (82)216 (72)
 Bulbar85 (18)84 (28)
FVC % predicted89±1569±14<0.001
ALSFRS-R total score38±534±6<0.001
ALSFRS-R dyspnoea score<0.001
 4316 (68)138 (46)
 3118 (25)87 (29)
 226 (6)48 (16)
 14 (1)27 (9)
 01 (<1)0 (0)
ALSFRS-R orthopnoea score<0.001
 4432 (93)225 (75)
 320 (4)23 (7)
 210 (2)32 (11)
 10 (0)2 (1)
 03 (1)18 (6)
Smoking history0.11
 Never256 (55)150 (50)
 Previous160 (34)125 (42)
 Current49 (11)25 (8)
Coronary artery disease34 (7)36 (12)0.028
Diabetes mellitus31 (7)50 (17)<0.001
Hypertension161 (35)136 (45)0.003

Data are presented as n (%), mean±sd, or median (interquartile range). Comparisons between data are made using the t-test, the Chi-squared test, or the Wilcoxon–Mann–Whitney test. BMI: body mass index; ALS: amyotrophic lateral sclerosis; FVC: forced vital capacity; ALSFRS-R: amyotrophic lateral sclerosis functional rating scale-revised.