Table 1

Clinical and demographic characteristics of research groups

C9+ ALSC9− ALS
n14156
Male, n (%)10 (71)107 (69)
Age at MRI, median (range)53.7 (40.6–67.0)61.8 (24.0–78.9)
Right-handed, n (%)*11 (92)129 (91)
ALSFRS-R, median (range)42 (33–47)42 (23–48)
Age at onset, median (range)52.9 (39.1–66.4)60.9 (23.5–78.1)
Bulbar onset, n (%)5 (36)41 (26)
Disease duration in months, median (range)12.3 (3.3–31.8)12.0 (3.7–75.7)
Rate of decline, median (range)†0.56 (0.15–1.14)0.46 (0.00–3.92)
FAB score, median (range)‡16 (13–18)17 (6–18)
VFI score, median (range)§12.9 (3.0–56.0)f6.7 (3.2–24.9)f
ALS-FTD, n (%)¶1 (7)1 (1)
Familial ALS, n (%)7 (50)f10 (6)f
Survival in months, median (95% CI)40.1 (28-NA)35.6 (31.6 to 44.2)
  • *Handedness in a subset of patients; C9+ ALS: n=12, C9− ALS: n=141.

  • †Points decrease of ALSFRS-R per month.

  • ‡FAB in a subset of patients (maximum score 18): C9+ ALS: n=9, C9− ALS: n=103.

  • §VFI in a subset of patients: C9+ ALS: n=8, C9− ALS: n=95.

  • ¶ALS-FTD according to the Neary criteria.

  • fp<0.01.

  • ALSFRS-R, ALS-Functional Rating Scale Revised; ALS-FTD, amyotrophic lateral sclerosis with comorbid frontotemporal dementia; FAB, Frontal Assessment Battery; NA, not available; VFI, Verbal Fluency Index.