Motor unit number estimation in patients with spinal muscular atrophy using the CMAP scan technique

Background: 5q-Spinal Muscular Atrophy (SMA) is one of the most prevalent neuromuscular diseases in our country, and still an important cause lethality, due to genetic disease, its severe forms. From a point view, it already known that number copies SMN2 gene drastically influences phenotype inverse relationship with severity disease. Several studies show reduction motor unit counts by different techniques. A new Motor Unit Number Estimation (MUNE) technique described 2016 Bostock, MScanfit (CMAP Scan MUNE), uses mathematical model considers stimulus-response curve compound muscle action potential (CMAP) estimate units. Studies have shown this has greater sensitivity than other conventional techniques (MUNIX MPS), addition not requiring voluntary activation, facilitating use children or patients marked weakness.