Phosphorylated TDP-43 (pTDP-43) aggregates in the axial skeletal muscle of patients with sporadic and familial amyotrophic lateral sclerosis

Table 2 Characteristics of muscle samples with and without pTDP-43 pathology

Variable	pTDP-43 Positive (n = 19)	pTDP-43 Negative (n = 38)	P-val ^a
Muscle group	17 Axial, 4 Appendicular ^b	56 Axial, 57 Appendicular ^b	0.003
Patient age	64 years (IQR, 58.5–69.5)	59.5 years (IQR, 54.3–66.5)	0.1
Disease duration	1114 days (IQR, 840–2134)	1085 days (IQR, 723–1390)	0.34
CNS TDP⁺ ROI ^c	58.3% TDP+ (IQR, 45.5–79.4)	50.0% TDP+ (IQR, 42.3–76.5)	0.67
Men, Women	13 men, 6 women	26 men, 12 women	NA
fALS, sALS	3 fALS, 16 sALS	7 fALS, 29 sALS	0.78
C9ALS ^d	4 C9ALS	9 C9ALS	0.80
Onset site	11 Limb, 5 Bulb, 3 Multifocal	27 Limb, 7 Bulb, 3 Multifocal	0.49

^aP-values represent differences using Fisher exact test or Mann-Whitney U test as appropriate (See Methods/ Results). ^b These numbers do not include three positive “NOS” samples, ten negative “NOS” samples, or negative abdominal wall muscle. ^c The percentage of brain and spinal cord ROIs positive for TDP-43 pathology as previously described in Cykowski et al., 2017 [17] (see Methods). ^d C9ALS status accounted for 9 of 10 fALS patients with one patient having an unknown genetic basis

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