Excitability of sensory axons in amyotrophic lateral sclerosis.


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 fallout, 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.

Matamala JM, Howells J, Dharmadasa J, Huynh W, Park SB, Burke D, Kiernan MC.

Clinical Neurophysioloy (Clin Neurophysiol.)

julio 01, 2018

DOI: 10.1016/j.clinph.2018.03.014

Investigador BNI: Dr. José Manuel Matamala (MD, PhD)