Table 1. Parameters in mitochondrial myopathy.
| 1 | 2 | 3 | 4 | 5 | |
|---|---|---|---|---|---|
| General | |||||
| Gender | F | M | M | M | M |
| Date of birth | 12.10.98 | 12.12.00 | 27.1.01 | 10.11.97 | 5.1.99 |
| Muscular | |||||
| Hypotonia | + | - | + | + | - |
| Muscle weakness | + | + | + | + | + |
| Muscle cramps | - | + | - | + | + |
| Exercise intolerance | + | + | + | + | + |
| Neurological | |||||
| Mental retardation | + | - | - | - | - |
| Epilepsy | - | - | - | - | - |
| Pyramidal involvement | - | - | - | - | - |
| Extrapyramidal symptoms | - | - | - | - | - |
| Visual loss | - | - | - | - | - |
| Hearing loss | - | - | - | - | - |
| Concentration problems | + | + | + | + | + |
| Multisystem | |||||
| Failure to thrive | - | + | - | - | - |
| Cardiac involvement | - | - | - | - | - |
| Renal involvement | - | - | - | - | - |
| Endocrine abnormalities | - | - | - | - | - |
| Chronic diarrhea | - | + | - | - | - |
| Food aversion | + | + | - | - | + |
| Biochemistry | |||||
| Complex deficiency | III + V | I + III | I | III | I |
| Alanin (blood) μmol/L | |||||
| (N<460) | 503 | 301–460 | 275–490 | 430–510 | 392–446 |
| Lactate (blood) mmol/L | |||||
| (N<2.1) | 0.9–2.7 | 1.1–3.8 | 1.1–1.8 | 1.4–1.9 | 1.3–3.2 |
| MDC score | 8 | 6 | 5 | 6 | 6 |
| Exercise tolorence | |||||
| MABC | 15 pc | 50 pc | 5 pc | 5–10 pc | 15 pc |
| Treadmill test 1* (Bruce17) | 8.55 min | 7 min | 11 min | 9.5 min | 9 min |
| Treadmill test 2** (Bruce17) | 8.44 min | 7.3 min | 10 min | 8 min | 9.1 min |
| VO2-max1+ | 45.6 | 51.05 | 48.18 | 49.92 | 51.2 |
| VO2-max2++ | 44.9 | 51.06 | 45.68 | 50.88 | 50.56 |
*
Exercise endurance according to Bruce17 at the time of first and last measurement;
+
VO2max at the first and last measurements.