Association between MR imaging clusters and clinical features in patients with ALSa

ALS Population (n = 90)Cluster 1 (n = 28)Cluster 2 (n = 51)Cluster 3 (n = 11)P Value
Age at onset (median) (IQR) (yr)57 (50–65)67 (62–70)41 (44–54)46 (45–54)P = .045b
P = .001c
P = nsd
Sex (No. of patients) (male/female)47:439:1929:229:2P  = .035b
P = .005c
P = nsd
Site of onset (spinal/bulbar) (No. of patients)67/2320/836/1511/0P = nsb
P  = .047c
P  = .039d
ALS phenotypes: classic/bulbar/flail arm/flail leg/pyramidal/respiratory/PLMN/PUMN (No. of patients)68/7/0/0/0/0/15/025/2/0/0/0/0/1/034/5//0/0/0/0/12/09/0//0/0/0/0/2/0P = nsb
P = nsc
P = nsd
El Escorial-revised, categories: definite/probable/possible (No. of patients)22/36/3212/14/28/18/252/4/5P < .001b
P = .017c
P = nsd
ODI (median) (IQR) (mo)10.07 (6.08–19.27)9.18 (6.08–14.32)11.73 (6.23–19.53)8.97 (4.07–19.27)P = nsb
P = nsc
P = nsd
ALSFRS-r (median score) (IQR)38 (34–42)34 (31–37)40 (35–43)40 (38–43)P  = .001b
P < .001c
P = nsd
Progression rate (median) (IQR)0.83 (0.45–1.38)1.26 (0.68–2.84)0.70 (0.29–1,20)0.80 (0.45–1.12)P  = .002b
P  = .022c
P = nsd
Long/short survivors (No. of patients)26/643/2517/346/5P  = .032b
P  = .008c
P = nsd
Survival time from symptom onset to composite outcome (median) (estimated median) (95% CI) (mo)46.63 (37.95–55.31)34.5 (22.39 −46.6)46.63 (35.4–57.87)72.73 (42.98–102.47)P = .043b
P  = .004c
P = nsd
  • Note:—ns indicates not significant; PLMN, prevalent lower motor neuron; PUMN, prevalent upper motor neuron.

  • a Group differences in the demographic and clinical variables were evaluated using a Mann-Whitney U test for continuous variables and χ2 tests for discrete variables. Log-rank tests were used to test for differences in survival between different radiologic clusters.

  • b Cluster 1 versus cluster 2.

  • c Cluster 1 versus cluster 3.

  • d Cluster 2 versus cluster 3.