Table 1

Cohort demographics and phenotypes

Total no of patients100
Age (years), mean (range)60.4 (22–87)
Site of first muscle weakness
 Upper limb40%
 Lower limb39%
Disease subtype
 LMN predominant12%
 UMN predominant5%
Family history of ALS7%
Time from onset of first muscle weakness (months), mean (SD, range)20.8 (20.4, 1–134)
Time from onset of first muscle weakness until death (months), mean (SD, range)*28.5 (21.0, 7–85)
ALSFRS-R, mean (range) at first assessment36.9 (15–47)
Revised El-Escorial criteria at first assessment
 Clinically probable laboratory supported36%
 Clinically probable30%
 Clinically suspected15%
 Clinically definite13%
 Clinically possible6%
  • ALS, amyotrophic lateral sclerosis; ALSFRS-R, revised ALS-Functional Rating Score; LMN, lower motor neuron; UMN, upper motor neuron.