Publication / Source: Neurodegenerative Disease Management
Authors: Jingsong Zhou & Jun Sun
Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig’s disease, is a fatal neuromuscular disorder characterized by progressive loss of motor neurons and severe skeletal muscle atrophy. Currently, there is no cure for ALS. Most patients die within 5 years after disease onset. The lifetime risk of ALS is about 1 in 472 in women and 1 in 350 in men . Treatment with the only US FDA approved drug, Riluzole, extends patient life span only for a few months with little improvement in ALS symptoms . Thus, there is an urgent need to further understand the pathogenic mechanisms in order to develop novel interventions for alleviating disease progression and improving the quality of life for ALS patients. Although the massive decline of the neuromuscular system is the most overwhelming phenomenon in ALS, other organs may also actively contribute to the progressive loss of neuromuscular function in the disease progression.