Abstract
In amyotrophic lateral sclerosis (ALS), respiratory muscle weakness causes ventilatory insufficiency and tissue hypoxia, which induces a number of metabolic pathways, and in particular increases erythropoietin (EPO) synthesis. EPO is a glycoprotein with neuroprotective properties that stimulates erythropoiesis. Here, EPO plasma level in a large population of ALS patients, with and without respiratory failure, was measured. Plasma EPO level of patients with ALS (n = 98), controls with other neuromuscular diseases (n = 58) and healthy controls (n = 20) has been quantified by ELISA. No significant difference was found between ALS patients and the two control groups. EPO level was not different between bulbar- and spinal-onset patients and was not correlated with disease duration or functional impairment. However, in the ALS group EPO level was higher in females (p = 0.0006) and correlated positively with age (p = 0.006). The subgroup of ALS patients with respiratory failure had higher plasma levels of EPO compared with ALS patients with preserved respiratory function (p = 0.016), but short-term non-invasive ventilation did not change EPO level. In conclusion, EPO levels were found to be significantly higher in ALS patients with respiratory impairment representing preservation of this homeostatic mechanism.
Acknowledgements
This work was supported by Fundação para a Ciência e Tecnologia, Portugal. We thank Michael Swash for helpful discussion.
Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.