Axonal Dysfunction Precedes Motor Neuronal Death in Amyotrophic Lateral Sclerosis.

ABSTRACT: Wide-spread fasciculations are a characteristic feature in amyotrophic lateral sclerosis (ALS), suggesting motor axonal hyperexcitability. Previous excitability studies have shown increased nodal persistent sodium conductances and decreased potassium currents in motor axons of ALS patients, both of the changes inducing hyperexcitability. Altered axonal excitability potentially contributes to motor neuron death in ALS, but the relationship of the extent of motor neuronal death and abnormal excitability has not been fully elucidated. We performed multiple nerve excitability measurements in the median nerve at the wrist of 140 ALS patients and analyzed the relationship of compound muscle action potential (CMAP) amplitude (index of motor neuronal loss) and excitability indices, such as strengt

SUBMITTER: Iwai Y 

PROVIDER: S-EPMC4934877 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature