Autonomic cardiovascular responses in distal myopathy (Welander)

Autonomically mediated cardiovascular responses were evaluated in 9 patients with Welander distal myopathy and compared to data from an age- and sex-matched control group. The myopathy patients had a normal respiratory sinus arrhythmia and a normal heart rate response to the Valsalva menoeuvre, indicating a normal vagal function. They had a normal initial heart rate response to the orthostatic position, indicating a normal function of the sympathetic nerves. The main difference between the groups was found in the orthostatic position. The myopathy patients reacted with a greater increase in systolic blood pressure and a smaller heart rate increase than the controls. This suggests an altered peripheral vasomotor function, possibly with a more predominant activation of alfa than beta adrenergic receptors leading to vasoconstriction. In addition, a low forearm blood flow at rest and a les pronounced blood flow increase during the isometric handgrip were found in the myopathy patients. This finding could also be explained by proneness to vasoconstriction. It is concluded that patients with Welander distal myopathy have no signs of dysfunction of the peripheral autonomic nerves.