Electrodiagnostic Studies in Clinical Trials for Motor Neuron Disease

Motor neuron disease (MND) is a group of neurodegenerative disorders characterized by death of upper motor neurons (UMN) and lower motor neurons(LMN). Clinical study of UMN loss is limited, but electrodiagnostic studies can be used readily to assess the functional state of LMN. Electrodiagnostic studies are regularly used in making the diagnosis of amyotrophic lateral sclerosis (ALS) and other forms of MND. They can also be used to follow disease progression and serve as end point measures in drug trials. In this role, electrodiagnostic studies can provide specific information not readily available from measurement of muscle strength or clinical functional scales. This article emphasizes the primary pathologic and secondary physiologic changes that take place after LMN loss and how they can be assessed by electrodiagnostic studies. It reviews the uses of routine electrodiagnostic studies for the diagnosis of MND but focuses on how special electrodiagnostic studies can be used as end point measures in drug trials.