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

Table 1 Demographic, clinical, and sample characteristics in 57 ALS patients

Characteristic	Entire cohort ^a
Age	61 years (IQR, 56–68), range 39–86 ^b
Duration	1090 days (IQR, 781–1565), range 315–4886
Men/ Women	39 men (68.4%)/ 18 women (31.6%)
fALS ^c/ sALS/ C9ALS	10 fALS (18.2%)/ 45 sALS (81.8%)/ 13 C9ALS
Onset site ^c	38 Limb (66.7%)/ 12 Bulbar (21.1%)/ 6 Multifocal (10.5%)
Axial muscle samples	44 Paraspinous (29.7%)/ 29 Diaphragm (19.6%)
Append. Muscle samples	34 Deltoid (22.9%)/ 26 Quadriceps (17.6%)
Other muscle samples	13 NOS ^d (8.8%)/ 1 gastrocnemius/ 1 abdominus rectus

^aData presented are medians with interquartile range (IQR) in parenthesis or number with percentage
^bFor the 17 non-ALS samples with atrophy studied, median age was 60 years (IQR, 51–62). For the 8 non-ALS paraspinous samples, median age was 62 years
^cfALS/ sALS and c9ALS status were not available in 2 patients. One patient had an unknown onset site
^dThese samples were obtained without reference to anatomic site and simply labeled as “muscle” in the original autopsy report

ISSN: 2051-5960