Table 3.
Exercise | ||||||||
---|---|---|---|---|---|---|---|---|
Rest | 0.5 min | 1.0 min | 1.5 min | 2.0 min | Exhaustion or 3 min | Recovery | ΔMax | |
MSNA (bursts min-1) | ||||||||
McArdle | 25 ± 5 | 28 ± 5 | 33 ± 6 | 44 ± 7 † | 44 ± 8 † | 47 ± 7 † | 31 ± 6 | 22 ± 6 † |
Controls | 20 ± 3 | 20 ± 4 | 24 ± 4 | 25 ± 5 | 23 ± 4 | 30 ± 4 | 19 ± 4 | 10 ± 2 |
McArdle + MADD | 22 | 34 | 34 | 41 | 46 | 41 | 25 | 24 |
MSNA (total activity) | ||||||||
McArdle | — | 25 ± 27 | 48 ± 22 | 130 ± 64 | 128 ± 77 † | 164 ± 71 † | 29 ± 15 † | 164 ± 71 † |
Controls | — | 0 ± 21 | 14 ± 16 | 22 ± 23 | 28 ± 25 | 59 ± 19 | 4 ± 22 | 59 ± 19 |
McArdle + MADD | — | 72 | 74 | 92 | 159 | 92 | 14 | 159 |
Heart rate (beats min-1) | ||||||||
McArdle | 70 ± 3 | 78 ± 5 | 81 ± 5 | 85 ± 5 | 89 ± 7 | 90 ± 6 | 75 ± 4 | 20 ± 3 |
Controls | 66 ± 2 | 84 ± 3 | 84 ± 2 | 86 ± 2 | 89 ± 2 | 92 ± 2 | 67 ± 4 | 25 ± 2 |
McArdle + MADD | 66 | 69 | 73 | 90 | 93 | 93 | 79 | 27 |
Mean BP (mmHg) | ||||||||
McArdle | 108 ± 12 | 130 ± 16 | 135 ± 17 | 142 ± 14 | 143 ± 14 | 160 ± 13 † | 132 ± 13 | 47 ± 7 † |
Controls | 122 ± 11 | 134 ± 12 | 142 ± 15 | 147 ± 14 | 155 ± 14 | 160 ± 13 | 125 ± 17 | 38 ± 4 |
McArdle + MADD | 117 | 134 | 138 | 152 | 160 | 160 | 138 | 43 |
Data are from five patients with myophosphorylase deficiency (McArdle), six healthy sedentary subjects (Controls) and one patient with combined myophosphorylase and myoadenylate deaminase deficiency (McArdle + MADD). MSNA is expressed both as bursts per min and total activity (% change from rest). ΔMax, maximal increase from Rest to Exercise. The separately shown results in the myophosphorylase- and myoadenylate-deficient patient are also integrated in the grouped data for all McArdle patients. Values are means ±s.e.m.
Significantly greater increase from rest in McArdle patients compared to controls (P <0.05).