Table 1. Clinical data on CTR, MND, T2DM and DM patients used in this study.
| Patient | Sex | Muscle | Age at biopsy | CTG repeat size | MRCa | MIRS [28]b | HOMA (<2,5)c | BMId | %EFe | PRf | QRSf |
|---|---|---|---|---|---|---|---|---|---|---|---|
| CTR-1 | F | BB | 58 | / | 127 | / | 1.7 | 22.7 | n.d. | n.d. | n.d. |
| CTR-2 | M | BB | 34 | / | 130 | / | 1.5 | 25.0 | n.d. | n.d. | n.d. |
| CTR-3 | F | BB | 32 | / | 128 | / | n.d. | 23.4 | n.d. | n.d. | n.d. |
| MND-1 | F | BB | 65 | / | 120 | / | n.d. | 17.6 | n.d. | n.d. | n.d. |
| MND-2 | M | VL | 43 | / | 114 | / | 2.3 | 24.2 | n.d. | n.d. | n.d. |
| MND-3 | M | VL | 64 | / | 130 | / | 1.8 | 22.6 | 67 | 184 | 102 |
| T2DM-1 | F | VL | 34 | / | 130 | / | 6.3 | 26.1 | n.d. | n.d. | n.d. |
| T2DM-2 | F | VL | 63 | / | 130 | / | 9.8 | 27.2 | n.d. | n.d. | n.d. |
| T2DM-3 | M | BB | 50 | / | 130 | / | 7.1 | 26.6 | n.d. | n.d. | n.d. |
| DM1-1 | F | TA | 39 | 300 | 125 | 4 | 1.3 | 29.93 | 54 | 192 | 130 |
| DM1-2 | F | TA | 34 | 250 | 126 | 3 | 1.2 | 20.44 | 64 | 174 | 86 |
| DM1-3 | F | TA | 24 | 300 | 129 | 2 | 2.4 | 20.2 | 52 | 233 | 102 |
| DM1-4 | F | TA | 36 | 630 | 112 | 4 | 1.6 | 20.58 | 58 | 158 | 92 |
| DM1-5 | F | TA | 34 | 550 | 124 | 3 | 1.4 | 21.2 | 56 | 186 | 94 |
| DM1-6 | M | BB | 56 | 230 | 120 | 3 | 5.3 | 26.1 | 54 | 208 | 102 |
| DM1-7 | M | TA | 24 | 800 | 129 | 5 | 2.3 | 28.3 | 50 | 176 | 102 |
| DM1-8 | F | TA | 22 | 250 | 126 | 2 | 2.5 | 19.71 | 68 | 166 | 90 |
| DM2-1 | F | BB | 61 | / | 122 | / | 1.9 | 24.6 | 69 | 160 | 72 |
| DM2-2 | F | BB | 36 | / | 128 | / | 1.3 | 21.4 | 70 | 164 | 79 |
| DM2-3 | M | BB | 49 | / | 125 | / | 2.4 | 24.4 | 87 | 156 | 87 |
CTR, control; MND, motor neuron disease; T2DM, type 2 diabetes mellitus, DM1, myotonic dystrophy type 1; DM2, myotonic dystrophy type 2; F, female; M, male; BB, biceps brachii; VL, vastus lateralis; TA, tibialis anterior.
aMedical Research Council, scale for muscle strength; scale (0–5 grade) on 13 muscles at both sides in the upper and lower limbs for a total of 130 maximum score.
bMuscle Impairment Rating Scale, stage of the disease in myotonic dystrophy type 1 (DM1) patients.
cHOMA (HOmeostasis Model Assessment) was calculated using the formula: HOMA = [glucose (mg/dl) x insulin (μU/Ml)/405], using fasting values.
dBody Mass Index.
eCardiac Ejection Fraction, normal values >50%.
fElectrocardiographic abnormalities: normal PR interval <200ms, normal QRS duration <100ms.