Levosimendan is in clinical development for the treatment of respiratory function in amyotrophic lateral sclerosis (ALS). ALS is a progressive disease of the nervous system, where nerve cells in the brain and spinal cord that control voluntary movement gradually deteriorate, causing loss of muscle function and paralysis. The gradual loss of neurons leads to a paralysing effect on muscles used for breathing, which usually leads to death from respiratory failure. Treatment options for patients with ALS are extremely limited with current treatment revolving around supporting breathing.
Levosimendan works through binding to a protein called troponin C, which sensitises cardiac and skeletal muscles to calcium and increases their force of contraction. This increased force of contraction is thought to increase diaphragm function and support respiratory dysfunction. Levosimendan is given as an oral capsule and if licensed, it will offer a treatment option for patients with ALS, potentially delaying the need for mechanical ventilation support.