Clinical Profiles and Patterns of Neurodegeneration in Amyotrophic Lateral Sclerosis: A Cluster-Based Approach Based on MR Imaging Metrics

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FIG 1.
Estimated marginal means with SDs of the PreCT (A), ParaCT (B), and MOv (C) are plotted for each radiologic cluster adjusted for age and sex. Estimated marginal means and SDs of the CS are reported in the black dotted lines. The asterisk indicates P < .001.
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FIG 2.
Kaplan Mayer survival curves in patients with ALS stratified according to radiologic clusters.

Association between MR imaging clusters and clinical features in patients with ALS^a

	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 = .045^b
					P = .001^c
					P = ns^d
Sex (No. of patients) (male/female)	47:43	9:19	29:22	9:2	P = .035^b
					P = .005^c
					P = ns^d
Site of onset (spinal/bulbar) (No. of patients)	67/23	20/8	36/15	11/0	P = ns^b
					P = .047^c
					P = .039^d
ALS phenotypes: classic/bulbar/flail arm/flail leg/pyramidal/respiratory/PLMN/PUMN (No. of patients)	68/7/0/0/0/0/15/0	25/2/0/0/0/0/1/0	34/5//0/0/0/0/12/0	9/0//0/0/0/0/2/0	P = ns^b
					P = ns^c
					P = ns^d
El Escorial-revised, categories: definite/probable/possible (No. of patients)	22/36/32	12/14/2	8/18/25	2/4/5	P < .001^b
					P = .017^c
					P = ns^d
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 = ns^b
					P = ns^c
					P = ns^d
ALSFRS-r (median score) (IQR)	38 (34–42)	34 (31–37)	40 (35–43)	40 (38–43)	P = .001^b
					P < .001^c
					P = ns^d
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 = .002^b
					P = .022^c
					P = ns^d
Long/short survivors (No. of patients)	26/64	3/25	17/34	6/5	P = .032^b
					P = .008^c
					P = ns^d
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 = .043^b
					P = .004^c
					P = ns^d

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.