TABLE 1

Baseline characteristics of the Penn cohort (n=765)

CharacteristicResult
Age at diagnosis years63±12
Male sex440 (58)
Ethnicity
 Caucasian662 (87)
 African-american53 (7)
 Other50 (6)
BMI class kg·m−2
 <18.530 (4)
 18.5–24.9318 (42)
 25–29.9270 (35)
 ≥30147 (19)
Diagnosis delay years1.0 (0.6–1.7)
El Escorial criteria
 Definite ALS152 (20)
 Possible ALS197 (26)
 Probable ALS239 (31)
 Suspected ALS177 (23)
Symptom onset site
 Limb596 (78)
 Bulbar169 (22)
FVC % predicted81±18
ALSFRS-R total score37±6
ALSFRS-R dyspnoea score
 4454 (59)
 3205 (27)
 274 (10)
 131 (4)
 01 (<1)
ALSFRS-R orthopnoea score
 4657 (86)
 343 (5)
 242 (5)
 12 (<1)
 021 (3)
Smoking history
 Current74 (10)
 Previous285 (37)
 Never406 (53)
Coronary artery disease70 (9)
Diabetes mellitus81 (11)
Hypertension297 (39)

Data are presented as n (%), mean±sd or median (interquartile range). BMI: body mass index; ALS: amyotrophic lateral sclerosis; FVC: forced vital capacity; ALSFRS-R: amyotrophic lateral sclerosis functional rating scale-revised.