Abstract

Objective: To evaluate the excitability of sensory axons in patients with amyotrophic lateral sclerosis (ALS). Methods: Comprehensive sensory nerve excitability studies were prospectively performed on 28 sporadic ALS patients, compared to age-matched controls. Sensory nerve action potentials were recorded from digit 2 following median nerve stimulation at the wrist. Disease severity was measured using motor unit number estimation (MUNE), the revised ALS Functional Rating Scale (ALSFRS-R) and the MRC scale. Results: There were no significant differences in standard and extended measures of nerve excitability between ALS patients and controls. These unchanged excitability measures included accommodation to long-lasting hyperpolarization and the threshold changes after two supramaximal stimuli during the recovery cycle. Excitability parameters did not correlate with MUNE, ALSFRS-R, APB MRC scale or disease duration. Conclusions: This cross-sectional study has identified normal axonal membrane properties in myelinated sensory axons of ALS patients. Previously described sensory abnormalities could be the result of axonal fall-out, possibly due to a ganglionopathy, or to involvement of central sensory pathways rostral to gracile and cuneate nuclei. Significance: These results demonstrate the absence of generalized dysfunction of the membrane properties of sensory axons in ALS in the face of substantial deficits in motor function. (C) 2018 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.