Table 1

Clinical characteristics for patients with amyotrophic lateral sclerosis (ALS) as a group and divided into three tertiles as defined by the rate of disease progression (ΔFS)

CharacteristicAll
n=164
ΔFS group 1 (<0.47)
n=56
ΔFS group 2 (0.47–1.11)
n=54
ΔFS group 3 (>1.11)
n=54
Gender (%)
 Male57556550
 Female43453550
Site of onset (%)
 Limb55665444
 Bulbar44334654
 Respiratory1202
Phenotype (%)
 ALS84748396
 ALS-FTD40120
 Flail-arm variant41002
 PBP4462
 PLS3800
 PMA1400
Median age at visit 164.7 (58, 73)63.3 (58, 71)64.7 (59, 75)69.4 (58, 75)
Symptom duration (years)1.0 (0.6, 2)2.2 (1.2, 4.9)1.0 (0.6, 1.5)0.6 (0.4, 0.9)
Median ALSFRS score visit 139 (33, 43)42 (36, 44)40 (36, 43)34 (26, 38)
Median ΔFS score at visit 10.67 (0.33, 1.47)0.24 (0.13, 0.34)0.67 (0.60, 0.94)2.69 (1.47, 4)
  • The median Amyotrophic Laterals Sclerosis Rating Scale-Revised (ALSFRS-R) was significantly lower in the highest tertile ΔFS group. Atypical ALS phenotypes such as primary bulbar palsy (PBP), primary lateral sclerosis (PLS) and progressive muscular atrophy (PMA) were infrequent, but more common in the lowest ΔFS group. All data is expressed as median (IQR).

  • FTD, frontotemporal dementia.