Table 1

Clinical comparison between groups of patients with a mutation in SOD1, TARDBP, FUS, hexanucleotide repeats in C9ORF72 and patients with no identified genetic defect (other FALS)

Patient groupsC9ORF72SOD1TARDBPFUSOther FALS
Age at onset (years)
 Mean (SE)58 (1)51 (2)52 (3)43 (2)60 (2)
 Median5751574061
 Range30–81 (n=120)21–73 (n=46)27–78 (n=18)25–68 (n=36)32–85 (n=70)
Disease duration (months)*
 Mean (SE)33 (2)81 (12)56 (8)34 (4)66 (12)
 Median2949482839
 Range3–85 (n=112)4–252 (n=44)20–156 (n=18)9–88 (n=32)8–336 (n=58)
Bulbar onset
 Ratio of patients48/1203/412/185/3514/69
FTD
 Ratio of patients27/670/272/181/205/30
Gender
 M:F ratio1.5:1 (n=130)2.1:1 (n=81)1.2:1 (n=56)1:1.7 (n=27)1.1:1 (n=40)
  • * Disease duration data included censored data (11 for C9ORF72, 8 for SOD1, 1 for TARDBP, 1 for FUS and 11 for other FALS).

  • The fraction represents the number of patients with bulbar onset/the total number of patients for whom site of onset information was available.

  • The fraction represents the number of patients with frontotemporal dementia (FTD)/the total number of patients who were examined for cognitive deficits.

  • n, number of patients with the information available.